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1. Passeron T, Thiam M, Colbachini P, Fall F: [First case of cutaneous plasmocytoma in Africa]. Dakar Med; 2001;46(2):155-6
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  • [Transliterated title] Premier cas de plasmocytome cutané africain.
  • Cutaneous plasmacytoma are dermo-hypodermic plasmacytic infiltrations.
  • At his third chemotherapy, he presented a pre-stemal nodule growing since 15 days.
  • Moreover it should make modify the therapeutic protocol, if it's possible.
  • [MeSH-major] Plasmacytoma. Skin Neoplasms

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  • (PMID = 15773188.001).
  • [ISSN] 0049-1101
  • [Journal-full-title] Dakar médical
  • [ISO-abbreviation] Dakar Med
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Senegal
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2. Horta P, Quevedo I: [Poems syndrome. Report of one case]. Rev Med Chil; 2004 Apr;132(4):485-8
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  • [Transliterated title] Síndrome de Poems: caso clínico.
  • Poems syndrome (polyneuropathy, organomegaly, endocrine abnormality, M-protein, plasma cell dyscrasia, and skin lesions) is a plasma cell dyscrasia whose pathogenesis is unknown.
  • The subsequent work up revealed an IgA type monoclonal gammopathy, hepatomegaly, hyperestrogenism, primary adrenal failure, hypothyroidism, hyperpigmentation and erythematous lesions in the skin and ankle edema.
  • [MeSH-minor] Anti-Inflammatory Agents / therapeutic use. Female. Humans. Immunoglobulin A / analysis. Middle Aged. Paraproteinemias / diagnosis. Paraproteinemias / drug therapy. Prednisone / therapeutic use

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  • (PMID = 15382521.001).
  • [ISSN] 0034-9887
  • [Journal-full-title] Revista médica de Chile
  • [ISO-abbreviation] Rev Med Chil
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Chile
  • [Chemical-registry-number] 0 / Anti-Inflammatory Agents; 0 / Immunoglobulin A; VB0R961HZT / Prednisone
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3. Silapunt S, Chon SY: Generalized necrobiotic xanthogranuloma successfully treated with lenalidomide. J Drugs Dermatol; 2010 Mar;9(3):273-6
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  • Necrobiotic xanthogranuloma (NXG) presents a therapeutic challenge to clinicians.
  • Generalized NXG has limited treatment options.
  • A patient presented to the authors with generalized NXG associated with monoclonal gammopathy of unknown significance (MGUS), a plasma cell dyscrasia considered to be a precursor to multiple myeloma.
  • The patient was treated with lenalidomide, a derivative of thalidomide with efficacy in treatment of multiple myeloma.
  • [MeSH-major] Granuloma / drug therapy. Necrobiotic Disorders / drug therapy. Thalidomide / analogs & derivatives
  • [MeSH-minor] Aged. Aged, 80 and over. Dexamethasone / therapeutic use. Humans. Male. Monoclonal Gammopathy of Undetermined Significance / drug therapy

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  • (PMID = 20232591.001).
  • [ISSN] 1545-9616
  • [Journal-full-title] Journal of drugs in dermatology : JDD
  • [ISO-abbreviation] J Drugs Dermatol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 4Z8R6ORS6L / Thalidomide; 7S5I7G3JQL / Dexamethasone; F0P408N6V4 / lenalidomide
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4. Sharabi Y, Raanani P, Shenkar A, Thaler M, Grossman E: Plasma cell dyscrasia with polyneuropathy--POEMS syndrome presenting with vasculitic skin lesions and responding to combination chemotherapy. Leuk Lymphoma; 2000 Dec;40(1-2):209-13
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  • [Title] Plasma cell dyscrasia with polyneuropathy--POEMS syndrome presenting with vasculitic skin lesions and responding to combination chemotherapy.
  • We report a 61-year-old male patient who presented with severe sensorimotor neuropathy, leg edema and skin lesions with M-paraprotein and 50% plasma cells in the bone marrow.
  • The patient was treated with aggressive combined chemotherapy, which induced improvement in both the clinical and laboratory parameters of his disease.
  • Our findings may shed light on the unknown pathogenesis of this syndrome and the successful results of treatment support the adoption of an aggressive therapeutic approach in symptomatic patients.
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Diagnosis, Differential. Humans. Male. Middle Aged. Paraproteinemias / drug therapy. Paraproteinemias / etiology. Paraproteinemias / pathology. Polyneuropathies / drug therapy. Polyneuropathies / etiology. Polyneuropathies / pathology. Vasculitis / etiology. Vasculitis / pathology

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  • (PMID = 11426623.001).
  • [ISSN] 1042-8194
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
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5. Rives S, Bladé J, Martínez C, Marín P, Carreras E, Montserrat E: [High-dose melphalan treatment followed by hematopoietic progenitor cell rescue in primary amyloidosis]. Med Clin (Barc); 2000 Jul 8;115(6):216-20
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  • [Title] [High-dose melphalan treatment followed by hematopoietic progenitor cell rescue in primary amyloidosis].
  • [Transliterated title] Tratamiento con melfalán a altas dosis seguido de rescate con progenitores hematopoyéticos en la amiloidosis primaria.
  • BACKGROUND: Primary systemic amyloidosis (AL) is a plasma cell dyscrasia characterized by the extracellular deposition of immunoglobulins light chains in different organs and systems.
  • Median survival with current standard treatment is less than 2 years.
  • Intensive chemotherapy followed by hematopoietic stem cell rescue has been used in AL for the last five years.
  • The reported results are encouraging, with a high response rate (65%).
  • Nonetheless, this procedure is associated to an important toxicity, with high transplant related mortality (about 25%).
  • In patients with advanced AL (more than two involved organs) and/or complicated cardiac disease, the mortality is particularly high.
  • In the current report we describe the outcome of the first five patients who received high dose therapy for AL at our institution.
  • PATIENTS AND METHODS: This treatment was administered to patients up to the age of 70 years, who met the standard eligibility criteria for an autologous bone marrow transplantation.
  • Intensive treatment consisted of melphalan 200 mg/m2 in all patients but one who received 140 mg/m2.
  • Both patients had a poor performance status and advanced disease, with more than two organs involved.
  • CONCLUSIONS: High dose therapy (HDT) with stem cell rescue in AL produces a high response rate.
  • Nevertheless, this treatment approach is associated to a high toxicity.
  • [MeSH-major] Amyloidosis / therapy. Antineoplastic Agents, Alkylating / administration & dosage. Hematopoietic Stem Cell Transplantation. Melphalan / administration & dosage
  • [MeSH-minor] Adult. Aged. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Male. Middle Aged. Postoperative Complications. Time Factors

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  • [ErratumIn] Med Clin (Barc) 2000 Oct 21;115(13):486
  • (PMID = 11002461.001).
  • [ISSN] 0025-7753
  • [Journal-full-title] Medicina clínica
  • [ISO-abbreviation] Med Clin (Barc)
  • [Language] spa
  • [Publication-type] Comparative Study; English Abstract; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] SPAIN
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; Q41OR9510P / Melphalan
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6. Orlowski RZ, Stinchcombe TE, Mitchell BS, Shea TC, Baldwin AS, Stahl S, Adams J, Esseltine DL, Elliott PJ, Pien CS, Guerciolini R, Anderson JK, Depcik-Smith ND, Bhagat R, Lehman MJ, Novick SC, O'Connor OA, Soignet SL: Phase I trial of the proteasome inhibitor PS-341 in patients with refractory hematologic malignancies. J Clin Oncol; 2002 Nov 15;20(22):4420-7
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  • In three of 10 patients receiving additional therapy, serious reversible adverse events appeared during cycle 2, including one episode of postural hypotension, one systemic hypersensitivity reaction, and grade 4 transaminitis in a patient with hepatitis C and a substantial acetaminophen ingestion.
  • PD studies revealed PS-341 induced 20S proteasome inhibition in a time-dependent manner, and this inhibition was also related to both the dose in milligrams per meter squared, and the absolute dose of PS-341.
  • Among nine fully assessable patients with heavily pretreated plasma cell dyscrasias completing one cycle of therapy, there was one complete response and a reduction in paraprotein levels and/or marrow plasmacytosis in eight others.
  • In addition, one patient with mantle cell lymphoma and another with follicular lymphoma had shrinkage of nodal disease.
  • [MeSH-major] Antineoplastic Agents / administration & dosage. Antineoplastic Agents / adverse effects. Boronic Acids / administration & dosage. Boronic Acids / adverse effects. Enzyme Inhibitors / administration & dosage. Enzyme Inhibitors / adverse effects. Hematologic Neoplasms / drug therapy. Multienzyme Complexes / antagonists & inhibitors. Pyrazines / administration & dosage. Pyrazines / adverse effects
  • [MeSH-minor] Adult. Aged. Bortezomib. Cysteine Endopeptidases. Drug Administration Schedule. Female. Humans. Male. Middle Aged. Proteasome Endopeptidase Complex. Treatment Outcome. United States

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  • (PMID = 12431963.001).
  • [ISSN] 0732-183X
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Grant] United States / NCRR NIH HHS / RR / RR00046
  • [Publication-type] Clinical Trial; Clinical Trial, Phase I; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Boronic Acids; 0 / Enzyme Inhibitors; 0 / Multienzyme Complexes; 0 / Pyrazines; 69G8BD63PP / Bortezomib; EC 3.4.22.- / Cysteine Endopeptidases; EC 3.4.25.1 / Proteasome Endopeptidase Complex
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7. Seckinger A, Meissner T, Moreaux J, Goldschmidt H, Fuhler GM, Benner A, Hundemer M, Rème T, Shaughnessy JD Jr, Barlogie B, Bertsch U, Hillengass J, Ho AD, Pantesco V, Jauch A, De Vos J, Rossi JF, Möhler T, Klein B, Hose D: Bone morphogenic protein 6: a member of a novel class of prognostic factors expressed by normal and malignant plasma cells inhibiting proliferation and angiogenesis. Oncogene; 2009 Nov 5;28(44):3866-79
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  • [Title] Bone morphogenic protein 6: a member of a novel class of prognostic factors expressed by normal and malignant plasma cells inhibiting proliferation and angiogenesis.
  • Pathogenesis of multiple myeloma is associated with an aberrant expression of pro-proliferative, pro-angiogenic and bone-metabolism-modifying factors by malignant plasma cells.
  • Given the frequently long time span from diagnosis of early-stage plasma cell dyscrasias to overt myeloma and the mostly low proliferation rate of malignant plasma cells, we hypothesize these to similarly express a novel class of inhibitory factors of potential prognostic relevance.
  • We assessed the expression of BMPs and their receptors by Affymetrix DNA microarrays (n=779) including CD138-purified primary myeloma cell samples (n=635) of previously untreated patients.
  • BMP6 is the only BMP expressed by malignant and normal plasma cells.
  • Its expression is significantly lower in proliferating myeloma cells, myeloma cell lines or plasmablasts.
  • BMP6 significantly inhibits the proliferation of myeloma cell lines, survival of primary myeloma cells and in vitro angiogenesis.
  • A high BMP6 expression in primary myeloma cell samples delineates significantly superior overall survival for patients undergoing high-dose chemotherapy independent of conventional prognostic factors (International Staging System (ISS) stage, beta(2) microglobulin).
  • [MeSH-major] Biomarkers, Tumor / biosynthesis. Bone Morphogenetic Protein 6 / biosynthesis. Cell Proliferation. Gene Expression Regulation, Neoplastic. Multiple Myeloma / metabolism. Multiple Myeloma / mortality. Neoplasm Proteins / biosynthesis. Neovascularization, Pathologic / metabolism. Neovascularization, Pathologic / mortality
  • [MeSH-minor] Disease-Free Survival. Female. Gene Expression Profiling. Humans. Male. Oligonucleotide Array Sequence Analysis. Plasma Cells. Survival Rate


8. Dispenzieri A, Gertz MA: Treatment of POEMS syndrome. Curr Treat Options Oncol; 2004 Jun;5(3):249-57
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  • [Title] Treatment of POEMS syndrome.
  • POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome is a rare multisystemic paraneoplastic syndrome driven by an underlying plasma cell dyscrasia.
  • Recognition of the complex of a combination of peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasmaproliferative disorder, skin changes, sclerotic bone lesions, Castleman disease, thrombocytosis, papilledema, peripheral edema, pleural effusions, ascites, fingernail clubbing, and white nails is the first step in effectively managing the disease.
  • Once a patient has been completely evaluated, each component of the disease should be addressed, while finalizing a treatment plan for the underlying plasma cell proliferative disorder.
  • In patients with a dominant sclerotic plasmacytoma, first-line therapy should include radiation to the lesion.
  • Retrospective analysis and personal experience would dictate that systemic therapy be considered for patients with diffuse sclerotic lesions or absence of any bone lesion and for patients who have not demonstrated stabilization of their disease 3 to 6 months after completing radiation therapy.
  • Treatments with demonstrated benefit include corticosteroids, low-dose alkylator therapy, and high-dose chemotherapy with peripheral blood stem cell transplant.
  • Until the pathogenesis is fully understood, these are the mainstays of treatment for patients with POEMS syndrome.
  • [MeSH-major] POEMS Syndrome / therapy
  • [MeSH-minor] Combined Modality Therapy. Decision Trees. Humans

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  • (PMID = 15115653.001).
  • [ISSN] 1527-2729
  • [Journal-full-title] Current treatment options in oncology
  • [ISO-abbreviation] Curr Treat Options Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 46
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9. Berenson JR, Anderson KC, Audell RA, Boccia RV, Coleman M, Dimopoulos MA, Drake MT, Fonseca R, Harousseau JL, Joshua D, Lonial S, Niesvizky R, Palumbo A, Roodman GD, San-Miguel JF, Singhal S, Weber DM, Zangari M, Wirtschafter E, Yellin O, Kyle RA: Monoclonal gammopathy of undetermined significance: a consensus statement. Br J Haematol; 2010 Jul;150(1):28-38
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  • On February 25, 2009, a panel of international experts on plasma cell dyscrasia and skeletal disease met to discuss monoclonal gammopathy of undetermined significance (MGUS).
  • This non-malignant B-cell disorder is the most common plasma cell dyscrasia and is associated with an increased risk of developing serious B-cell disorders.
  • The panel also addressed the identification and treatment of MGUS-related skeletal problems, thromboembolic events and neurological complications.
  • The following consensus statement outlines the conclusions and marks the first time that a consensus statement for the screening and treatment of MGUS has been clearly stated.
  • [MeSH-minor] Aged. Bone Diseases, Metabolic / diagnosis. Bone Diseases, Metabolic / etiology. Cell Transformation, Neoplastic. Disease Progression. Humans. Long-Term Care / methods. Long-Term Care / standards. Mass Screening / methods. Mass Screening / organization & administration. Middle Aged. Peripheral Nervous System Diseases / drug therapy. Peripheral Nervous System Diseases / etiology. Prognosis. Risk Factors

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  • (PMID = 20507313.001).
  • [ISSN] 1365-2141
  • [Journal-full-title] British journal of haematology
  • [ISO-abbreviation] Br. J. Haematol.
  • [Language] eng
  • [Publication-type] Consensus Development Conference; Journal Article
  • [Publication-country] England
  • [Number-of-references] 86
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10. Harnalikar M, Pande S, Kharkar V, Khopkar U: Keratotic vascular papules over the feet: a case of Waldenström's macroglobulinaemia-associated cutaneous macroglobulinosis. Clin Exp Dermatol; 2010 Apr;35(3):278-81
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  • Waldenström's macroglobulinaemia (WM) is a plasma-cell dyscrasia characterized by the monoclonal proliferation of lymphoplasmacytes.
  • A 48-year-old man presented with a 4-year history of multiple painful, hyperkeratotic deep-seated papules over the pressure areas of both soles.
  • The patient received five cycles of chemotherapy, resulting in nearly complete resolution of the skin lesions and systemic symptoms.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Foot Diseases / pathology. Keratosis / pathology. Waldenstrom Macroglobulinemia / complications
  • [MeSH-minor] Chlorambucil / therapeutic use. Cyclophosphamide / therapeutic use. Diagnosis, Differential. Fluorescent Antibody Technique, Direct. Humans. Immunoglobulin M / blood. Male. Middle Aged. Treatment Outcome. Vidarabine / analogs & derivatives. Vidarabine / therapeutic use

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  • (PMID = 19874364.001).
  • [ISSN] 1365-2230
  • [Journal-full-title] Clinical and experimental dermatology
  • [ISO-abbreviation] Clin. Exp. Dermatol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Immunoglobulin M; 18D0SL7309 / Chlorambucil; 8N3DW7272P / Cyclophosphamide; FA2DM6879K / Vidarabine; P2K93U8740 / fludarabine
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11. Jaccard A, Royer B, Bordessoule D, Brouet JC, Fermand JP: High-dose therapy and autologous blood stem cell transplantation in POEMS syndrome. Blood; 2002 Apr 15;99(8):3057-9
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  • [Title] High-dose therapy and autologous blood stem cell transplantation in POEMS syndrome.
  • We treated 5 patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome and multifocal bone lesions or diffuse bone marrow plasmacytic infiltration with high-dose therapy (HDT) and autologous blood stem cell transplantation.
  • In all cases, the treatment produced remission of plasma cell proliferation associated with marked improvement in the patients' performance status, neurologic symptoms, and other manifestations of the syndrome.
  • HDT with stem cell support should be investigated further as a therapeutic option in patients with POEMS syndrome and disseminated plasma cell dyscrasia.
  • [MeSH-major] Antineoplastic Agents, Alkylating / administration & dosage. Hematopoietic Stem Cell Transplantation. POEMS Syndrome / therapy
  • [MeSH-minor] Adult. Clone Cells / immunology. Clone Cells / pathology. Combined Modality Therapy. Dose-Response Relationship, Drug. Female. Humans. Immunoglobulins / blood. Immunoglobulins / urine. Male. Melphalan / administration & dosage. Middle Aged. Nervous System Diseases / etiology. Plasma Cells / immunology. Plasma Cells / pathology. Remission Induction. Transplantation, Autologous. Treatment Outcome

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  • (PMID = 11929800.001).
  • [ISSN] 0006-4971
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 0 / Immunoglobulins; Q41OR9510P / Melphalan
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12. Dumesnil C, Schneider P, Dolgopolov I, Radi S, Leluyer B, Vannier JP: Solitary bone plasmocytoma of the spine in an adolescent. Pediatr Blood Cancer; 2006 Sep;47(3):335-8
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  • Solitary plasmocytoma (SP) represent only about 5% of plasma cell neoplasia.
  • Most patients have generalized disease, that is, multiple myeloma (MM).
  • Solitary bone plasmocytoma (SBP) is a localized plasma cell tumor and is a very rare disease in young patients.
  • We reported here, a case of SPB in a 14-year-old girl with a 10-year disease-free survival after an aggressive treatment.
  • MRI of the spine is necessary to assess local disease.
  • Radiotherapy with doses of 45-50 Gy is the recommended treatment and gives a high rate of local control (83-96%).
  • Chemotherapy remains controversial in contrast to MM, in which intensive chemotherapy with autologous bone marrow transplantation (ABMT) is widely accepted.
  • At the present time, considering the good prognosis of patients with a normal MRI at diagnosis and a complete disappearance of the M protein after radiotherapy, we believe that ABMT should be reserved for relapse or primary therapeutic failure.
  • [MeSH-major] Bone Neoplasms / pathology. Plasmacytoma / pathology. Spinal Neoplasms / pathology
  • [MeSH-minor] Adolescent. Disease-Free Survival. Female. Humans. Magnetic Resonance Imaging. Neoplasm Recurrence, Local / diagnosis. Neoplasm Recurrence, Local / pathology. Neoplasm Recurrence, Local / therapy. Prognosis. Sensitivity and Specificity. Treatment Outcome

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  • (PMID = 16086417.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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13. Tazawa K, Matsuda M, Yoshida T, Gono T, Katoh N, Shimojima Y, Ishii W, Fushimi T, Koyama J, Ikeda S: Therapeutic outcome of cyclic VAD (vincristine, doxorubicin and dexamethasone) therapy in primary systemic AL amyloidosis patients. Intern Med; 2008;47(17):1517-22
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  • [Title] Therapeutic outcome of cyclic VAD (vincristine, doxorubicin and dexamethasone) therapy in primary systemic AL amyloidosis patients.
  • OBJECTIVE: Intensive chemotherapy targeting plasma cell dyscrasia has been recently employed for the treatment of primary systemic AL amyloidosis.
  • We prospectively studied the clinical usefulness of cyclic VAD (vincristine, doxorubicin and dexamethasone) in patients with primary systemic AL amyloidosis who were ineligible for high-dose melphalan with autologous stem cell support.
  • RESULTS: Four patients (50%) showed a marked decrease in abnormal plasma cells in the bone marrow and normalized kappa/lambda ratios of serum free light chain in conjunction with disappearance of M-protein after 1 to 3 courses of VAD.
  • CONCLUSION: Cyclic VAD is a potent therapeutic option in primary systemic AL amyloidosis, but in patients with renal or cardiac dysfunction careful management for adverse events, especially body fluid retention, is necessary.
  • [MeSH-major] Amyloidosis / drug therapy. Amyloidosis / mortality. Antineoplastic Combined Chemotherapy Protocols / administration & dosage
  • [MeSH-minor] Aged. Dexamethasone / administration & dosage. Doxorubicin / administration & dosage. Female. Follow-Up Studies. Humans. Male. Middle Aged. Myeloma Proteins / antagonists & inhibitors. Prospective Studies. Survival Rate / trends. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 18758127.001).
  • [ISSN] 1349-7235
  • [Journal-full-title] Internal medicine (Tokyo, Japan)
  • [ISO-abbreviation] Intern. Med.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Myeloma Proteins; 0 / multiple myeloma M-proteins; 5J49Q6B70F / Vincristine; 7S5I7G3JQL / Dexamethasone; 80168379AG / Doxorubicin; VAD protocol
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14. Schönland SO, Bochtler T, Kristen AV, Ho AD, Hegenbart U: [Current diagnostic and therapy of light chain amyloidosis]. Pathologe; 2009 May;30(3):205-11
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  • [Title] [Current diagnostic and therapy of light chain amyloidosis].
  • [Transliterated title] Aktuelle Diagnostik und Therapie der Leichtkettenamyloidose.
  • Amyloidoses are protein-folding disorders in which soluble proteins are deposited as insoluble fibrillar aggregates due to a change in protein conformation.
  • The light chain type is the most common form of systemic amyloidoses and has the worst prognosis.
  • The underlying disease is a monoclonal, mostly non-malignant plasma cell disorder.
  • The causative treatment is the reduction of the amyloidogenic light chains with conventional or high-dose chemotherapy.
  • Meanwhile, the"new drugs" used in multiple myeloma are also successfully applied.
  • Patients with newly diagnosed amyloidosis should be referred to a specialized center for consultation, diagnosis and treatment recommendation.
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Bone Marrow / pathology. Diagnosis, Differential. Dose-Response Relationship, Drug. Early Diagnosis. Humans. Multiple Myeloma / drug therapy. Multiple Myeloma / pathology. Plasma Cells / pathology. Protein Folding / drug effects. Referral and Consultation

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  • (PMID = 19343349.001).
  • [ISSN] 1432-1963
  • [Journal-full-title] Der Pathologe
  • [ISO-abbreviation] Pathologe
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Amyloid; 0 / Antineoplastic Agents; 0 / Immunoglobulin Light Chains; 0 / amyloid protein AL
  • [Number-of-references] 29
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15. Ishikawa H, Tsuyama N, Mahmoud MS, Fujii R, Abroun S, Liu S, Li FJ, Kawano MM: CD19 expression and growth inhibition of tumours in human multiple myeloma. Leuk Lymphoma; 2002 Mar;43(3):613-6
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  • Multiple myeloma (MM) is a proliferative disorder of monoclonal plasma cells which accumulate in human bone marrow (BM).
  • CD19 is a hallmark differentiation antigen of the B cell lineage and positively regulates antigen receptor signal transduction in mature B cells.
  • We have previously shown that malignant plasma cells (myeloma cells) isolated from the MM patients lack the CD19 expression, while non-malignant plasma cells isolated from the healthy donors do express the CD19 antigens.
  • It is also intriguing that there exists both CD19- and CD19+ plasma cells in some cases in pre-myeloma states including monoclonal gammopathy of undetermined significance (MGUS).
  • It indicates that MGUS is usually composed of phenotypically non-malignant (CD19+) and malignant (CD19-) plasma cells.
  • Furthermore, we recently demonstrate that, expression of the CD19 gene markedly inhibits the proliferation of human myeloma cell lines in vitro, and exhibits the reduced tumorigenicity in vivo and no anchorage-independent growth in vitro of a tumorigenic myeloma cell line.
  • This inhibitory effect might result from the CD19-mediated intracellular signals because it is not observed in cells expressing the mutant CD19, which lacks the cytoplasmic domain.
  • In this review, we suggest that loss of CD19 in MM could contribute to the proliferative advantage of the malignant plasma cell clones in this disease.
  • Furthermore, we propose the usefulness of the phenotypic analysis of plasma cells in human plasma cell dyscrasia as a new diagnostic tool, and the CD19 gene as a potential target for the gene therapy in MM.
  • [MeSH-minor] Cell Division / drug effects. Humans. Plasma Cells / cytology. Plasma Cells / metabolism. Plasma Cells / pathology

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  • (PMID = 12002767.001).
  • [ISSN] 1042-8194
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Antigens, CD19
  • [Number-of-references] 27
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16. Trinn C: [Amyloidosis]. Orv Hetil; 2010 Jul 18;151(29):1182-9
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  • The term amyloidosis refers to the extracellular deposition of fibrils composed of different types of plasma proteins.
  • Various clinical symptoms are caused by the tissue damage related to the deposited fibrillary material.
  • Scintigraphy with radioisotope labeled serum amyloid P-component is helpful in the localization of the process and in the assessment of therapeutic effect.
  • In the majority of cases the underlying disease is a plasma cell disorder, light chains aggregate to amyloid fibrils.
  • Therefore chemotherapy and - in selected patients - stem cell transplantation is the choice of treatment.
  • Another common type of amyloidosis is caused by chronic inflammatory diseases (amyloid fibrils are composed of elevated serum amyloid A being related to C reactive protein), or by some hereditary fever syndromes.
  • Treatment of the underlying disorder may bring resolution of the amyloid burden.
  • Depending on the source of the mutant protein liver transplantation, hepatorenal or cardiorenal transplantation may cure the disease.

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  • (PMID = 20591787.001).
  • [ISSN] 0030-6002
  • [Journal-full-title] Orvosi hetilap
  • [ISO-abbreviation] Orv Hetil
  • [Language] hun
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Hungary
  • [Chemical-registry-number] 0 / Biomarkers; 0 / Serum Amyloid A Protein
  • [Number-of-references] 22
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17. Bayer-Garner IB, Smoller BR: The spectrum of cutaneous disease in multiple myeloma. J Am Acad Dermatol; 2003 Apr;48(4):497-507
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The spectrum of cutaneous disease in multiple myeloma.
  • BACKGROUND: Multiple myeloma (MM) is a plasma cell dyscrasia characterized by a clonal proliferation of plasma cells that produces a monoclonal protein.
  • There are dermatologic disorders that have been associated with MM, such as amyloidosis, cryoglobulinemia, POEMS syndrome, normolipemic plane xanthoma, and plasmacytoma.
  • DESIGN: We reviewed 2357 pathology reports of all patients with a diagnosis of MM to find those who had undergone a skin biopsy.
  • Files were searched for bone-marrow diagnosis, and for type and number of transplants.
  • Skin biopsy specimen diagnoses included neoplastic lesions, (111; 73 malignant, 38 benign), graft-versus-host disease (120), drug-related lesions (46), cutaneous eruption of lymphocyte recovery (3), thrombocytopenia-related lesions (9), normolipemic plane xanthoma (1), amyloidosis (1), Sweet's syndrome (7), panniculitis (1), papulosquamous lesions (18), bullous diseases (17), vasculitis (11), infectious lesions (41), granulomatous dermatitis (6), alopecia cicatrisata (1), nonspecific lesions (77), and unrelated lesions (2).
  • CONCLUSIONS: Skin biopsy specimens from patients with MM less than 60 days from transplant most commonly show sequelae of the transplant such as graft-versus-host disease, Grover's disease (as a result of leukocytopenia and fever, waiting for engraftment), drug eruptions, chemotherapy effect, thrombocytopenic effect, cutaneous eruption of lymphocyte recovery, and Sweet's syndrome (possibly as a result of granulocyte-macrophage colony-stimulating factor).
  • Biopsy specimens taken more than 60 days from transplant most commonly show graft-versus-host disease, drug eruptions, and Sweet's syndrome but also show unrelated conditions such as neoplastic lesions, nevi, papulosquamous lesions, vasculitis, infections, and nonspecific changes.
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Biopsy. Bone Marrow Transplantation / adverse effects. Female. Graft vs Host Disease / pathology. Humans. Male. Middle Aged. Skin / pathology. Skin Neoplasms / pathology

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  • (PMID = 12664010.001).
  • [ISSN] 0190-9622
  • [Journal-full-title] Journal of the American Academy of Dermatology
  • [ISO-abbreviation] J. Am. Acad. Dermatol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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18. Soubrier M: [POEMS syndrome]. Presse Med; 2007 Nov;36(11 Pt 2):1676-82
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • The POEMS syndrome combines a constant polyneuropathy (P), organomegaly (O), endocrinopathy (E), monoclonal gammopathy (M) (or other plasma cell disorder) and skin changes (S).
  • Its pathogenesis is not well elucidated but elevated levels of vascular endothelial growth factor (VEGF) appear to characterize it.
  • Consistent plasma cell disorders include a monoclonal component, often in small quantities with a lambda light chain isotype, and plasmacytoma, often solitary lesions.
  • Treatment depends on specific characteristics of the disease and the patient (radiation therapy for plasmocytoma, autologous bone marrow transplantation in young subjects, corticosteroid therapy or chemotherapy in the elderly).

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  • (PMID = 17629447.001).
  • [ISSN] 0755-4982
  • [Journal-full-title] Presse medicale (Paris, France : 1983)
  • [ISO-abbreviation] Presse Med
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Number-of-references] 28
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19. Wilson KS: Regression of follicular lymphoma with Devil's Claw: coincidence or causation? Curr Oncol; 2009 Aug;16(4):67-70
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  • BACKGROUND: Cancer patients frequently use alternative therapies.
  • Two follicular lymphoma patients who had objective tumour regression after taking Devil's Claw without cytotoxic therapy are reported here.
  • METHODS AND RESULTS: Patient 1 presented with coexistent immunoglobulin G plasma cell dyscrasia and stage iiia lymphoma (nodes 5 cm in diameter).
  • Computed tomography scan 10 months later showed partial regression.
  • This patient later developed overt myeloma, at which time he stopped the herbal supplements and underwent high-dose chemotherapy and stem cell transplantation, since which no lymphoma progression has occurred.
  • Computed tomography scan 11 months later showed decreased adenopathy and splenomegaly, which has been sustained for 4 years.
  • Inhibition of COX-2 has a role in colon cancer prevention, has been implicated in lymphomagenesis, and is associated both with lymphoma stage and with response to treatment.
  • However, spontaneous regression in lymphoma has been reported in 16% of patients in one series, of whom none were on herbal medications or COX-2 inhibitors.
  • The key issue in both these patients is whether disease regression was "spontaneous" or causally related to therapy with Devil's Claw.
  • Further investigation is warranted, preferably with a COX-2 inhibitor of known purity.

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  • (PMID = 19672427.001).
  • [ISSN] 1198-0052
  • [Journal-full-title] Current oncology (Toronto, Ont.)
  • [ISO-abbreviation] Curr Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Canada
  • [Other-IDs] NLM/ PMC2722058
  • [Keywords] NOTNLM ; Low-grade lymphoma / alternative therapy / cox-2 inhibition
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20. Jimenez-Zepeda VH, Domínguez-Martínez VJ: Vincristine, doxorubicin, and dexamethasone or thalidomide plus dexamethasone for newly diagnosed patients with multiple myeloma? Eur J Haematol; 2006 Sep;77(3):239-44
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  • Multiple myeloma (MM) is a malignant plasma cell tumor that is distributed at multiple sites within the bone marrow compartments.
  • High-dose dexamethasone regimens [including vincristine, doxorubicin, and dexamethasone (VAD) chemotherapy] induce rapid responses, and have resulted in improved survival for many patients when followed by intensive therapy with autologous stem cell support early in the disease course.
  • P = 0.0005. In summary, we conclude Thal/dex is an effective therapy in newly diagnosed MM inducing objective responses in over 84.3%.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Multiple Myeloma / drug therapy

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  • (PMID = 16856924.001).
  • [ISSN] 0902-4441
  • [Journal-full-title] European journal of haematology
  • [ISO-abbreviation] Eur. J. Haematol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Comparative Study; Journal Article
  • [Publication-country] Denmark
  • [Chemical-registry-number] 4Z8R6ORS6L / Thalidomide; 5J49Q6B70F / Vincristine; 7S5I7G3JQL / Dexamethasone; 80168379AG / Doxorubicin; VAD I protocol
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21. Lizis-Kolus K, Kowalska A, Nowakowska-Domagała M: [The POEMS syndrome with coexisting endocrinopathy--case report]. Endokrynol Pol; 2007 May-Jun;58(3):238-43
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  • We present a case of a woman with unique multisystem disorder--POEMS syndrome and endocrine abnormalities coexisting with it.
  • Association between plasma cell dyscrasia and polyneuropathy, was described in 1956 year by Crow.
  • Within 3 years of the first symptoms, she developed hypogonadism hypergonadotropic.
  • Due to the progression of the disease, a thalidomide was used in therapy (it is anti-VEGF agent).
  • One of the side effects of the treatment of thalidomide is the progression of polyneuropathy, which was observed in this patient.
  • After finishing this therapy she received chemotherapy.
  • This case report imposes the necessity of constants observation of patients with POEMS syndrome because there is a possibility of their developing other disorders.
  • In the event of coexistence polyneuropathy and plasma cell dyscrasia, this disease should be taken into consideration.

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  • (PMID = 17940990.001).
  • [ISSN] 0423-104X
  • [Journal-full-title] Endokrynologia Polska
  • [ISO-abbreviation] Endokrynol Pol
  • [Language] pol
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Poland
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22. Sjak-Shie NN, Vescio RA, Berenson JR: Recent advances in multiple myeloma. Curr Opin Hematol; 2000 Jul;7(4):241-6
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  • Our understanding of the pathophysiology underlying myeloma continues to expand, but the cause of this plasma cell dyscrasia remains unclear.
  • The roles of cytogenetic abnormalities as well as aberrant angiogenesis and cytokine expression in the etiology of myeloma continue to be explored and may lead to future therapeutic strategies.
  • Transplantation in myeloma is rarely curative but offers clinical benefit not only for young but possibly for older myeloma patients as well.
  • Finally, thalidomide offers significant clinical benefit to patients with myeloma previously refractory to multiple agents, and its role in early stages of the disease is under investigation.
  • [MeSH-major] Multiple Myeloma / therapy
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Marrow / pathology. Bone Marrow / virology. Bone Marrow Transplantation. Chromosome Aberrations. Combined Modality Therapy. Cytokines / physiology. Diphosphonates / therapeutic use. Growth Substances / physiology. Hematopoietic Stem Cell Transplantation. Herpesviridae Infections / complications. Herpesviridae Infections / diagnosis. Herpesvirus 8, Human / isolation & purification. Herpesvirus 8, Human / pathogenicity. Humans. Neoplasm Proteins / physiology. Neovascularization, Pathologic. Osteolysis / drug therapy. Osteolysis / etiology. Osteolysis / radiotherapy. Plasma Cells / pathology. Remission Induction. Salvage Therapy. Thalidomide / therapeutic use

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  • (PMID = 10882180.001).
  • [ISSN] 1065-6251
  • [Journal-full-title] Current opinion in hematology
  • [ISO-abbreviation] Curr. Opin. Hematol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / Cytokines; 0 / Diphosphonates; 0 / Growth Substances; 0 / Neoplasm Proteins; 4Z8R6ORS6L / Thalidomide
  • [Number-of-references] 75
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23. Takahashi R, Nakano S, Namura K, Yamada N, Uchida R, Fuchida S, Okano A, Okamoto M, Ochiai N, Shimazaki C: Plasmacytoma of the urinary bladder in a renal transplant recipient. Int J Hematol; 2005 Apr;81(3):255-7
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  • After the reduction of immunosuppressive therapy, she received combined chemotherapy, resulting in complete tumor disappearance.
  • However, 10 months later, she relapsed with aggressive multiple EMP and died of disease progression in 2003.
  • This report is the first of a case of solitary EMP of the urinary bladder appearing as posttransplantation plasma cell dyscrasias after renal transplantation.
  • [MeSH-major] Kidney Transplantation. Neoplasm Recurrence, Local / pathology. Plasmacytoma / pathology. Urinary Bladder Neoplasms / pathology

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  • (PMID = 15814337.001).
  • [ISSN] 0925-5710
  • [Journal-full-title] International journal of hematology
  • [ISO-abbreviation] Int. J. Hematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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24. Nonami A, Miyamoto T, Kuroiwa M, Kunisaki Y, Kamezaki K, Takenaka K, Harada N, Teshima T, Harada M, Nagafuji K: Successful treatment of primary plasma cell leukaemia by allogeneic stem cell transplantation from haploidentical sibling. Jpn J Clin Oncol; 2007 Dec;37(12):969-72
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  • [Title] Successful treatment of primary plasma cell leukaemia by allogeneic stem cell transplantation from haploidentical sibling.
  • Primary plasma cell leukaemia (PCL) is a rare, aggressive neoplasm of plasma cell dyscrasia.
  • Conventional chemotherapy is usually ineffective, with an overall survival of only 8 months.
  • Here, we describe a 42-year-old man with primary PCL, who was successfully treated with haploidentical (2-HLA loci mismatched) haematopoietic stem-cell transplantation (HSCT).
  • To overcome the human leukocyte antigen (HLA) disparity, in vivo T-cell purging by the pre-transplant administration of antithymocyte globulin followed by a conventional prophylactic treatment against graft-versus-host disease (GVHD) resulted in an avoidance of severe GVHD as well as infectious complications.
  • Haploidentical HSCT can be a potentially curative treatment for patients with primary PCL who do not have an HLA-identical donor.
  • [MeSH-major] Haplotypes. Hematopoietic Stem Cell Transplantation. Leukemia, Plasma Cell / surgery. Siblings. Transplantation Conditioning / methods
  • [MeSH-minor] Adult. Antilymphocyte Serum / administration & dosage. Bone Marrow Purging. Graft vs Host Disease / prevention & control. Humans. Immunosuppressive Agents / administration & dosage. Male. T-Lymphocytes. Transplantation, Homologous. Treatment Outcome

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  • (PMID = 18055567.001).
  • [ISSN] 1465-3621
  • [Journal-full-title] Japanese journal of clinical oncology
  • [ISO-abbreviation] Jpn. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antilymphocyte Serum; 0 / Immunosuppressive Agents
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25. Sonneveld P, Segeren CM: Changing concepts in multiple myeloma: from conventional chemotherapy to high-dose treatment. Eur J Cancer; 2003 Jan;39(1):9-18
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  • [Title] Changing concepts in multiple myeloma: from conventional chemotherapy to high-dose treatment.
  • The treatment of Multiple Myeloma (MM), a malignant plasma cell disorder has changed considerably over the past decade.
  • It has been convincingly shown that intensive treatment supported by autologous stem cell reinfusion is superior to conventional chemotherapy with alkylating agents or vincristine, doxorubicin and dexamethasone (VAD) alone in terms of a more rapid response and a longer disease-free survival.
  • However, cure is not achieved in the majority of patients.
  • Several trials have therefore focussed on repeated intensive treatments in order to improve the survival of these patients.
  • Other approaches are aimed at identifying patients on the basis of prognostic factors, who may benefit from high-dose therapy.
  • This review discusses the recent developments in intensive therapy for multiple myeloma.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Hematopoietic Stem Cell Transplantation / methods. Multiple Myeloma / therapy

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  • (PMID = 12504653.001).
  • [ISSN] 0959-8049
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; Q41OR9510P / Melphalan
  • [Number-of-references] 88
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26. Liu YC, Miyazawa K, Sashida G, Kodama A, Ohyashiki K: Deletion (20q) as the sole abnormality in Waldenström macroglobulinemia suggests distinct pathogenesis of 20q11 anomaly. Cancer Genet Cytogenet; 2006 Aug;169(1):69-72
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  • [Title] Deletion (20q) as the sole abnormality in Waldenström macroglobulinemia suggests distinct pathogenesis of 20q11 anomaly.
  • The deletion of the long arm of chromosome 20, or del(20q), is a common cytogenetic abnormality in various myeloid disorders but is less commonly seen in lymphoid neoplasms.
  • Reviewing all 11 reported cases of plasma cell dyscrasia possessing sole del(20q), including our case, none of 4 cases with del(20q) as an initial anomaly developed myelodysplastic syndrome-acute myeloid leukemia (MDS/AML), but at least 3 cases with del(20q) appearing after chemotherapy developed MDS/AML at or after the time of del(20q).
  • We propose that the del(20q) may have different clinical significance in plasma cell dyscrasia: one is when del(20q) appears at diagnosis and may involve the initial event of oncogenesis, and the other is when del(20q) appears after treatment and is associated with therapy-related and potential MDS/AML risk.


27. Dispenzieri A, Lacy MQ, Zeldenrust SR, Hayman SR, Kumar SK, Geyer SM, Lust JA, Allred JB, Witzig TE, Rajkumar SV, Greipp PR, Russell SJ, Kabat B, Gertz MA: The activity of lenalidomide with or without dexamethasone in patients with primary systemic amyloidosis. Blood; 2007 Jan 15;109(2):465-70
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  • Primary systemic amyloidosis (AL) is an incurable plasma cell disorder.
  • Patients with symptomatic AL, a measurable plasma cell disorder, and adequate hematologic and renal reserve were eligible.
  • Within the first 3 cycles of therapy, 10 patients discontinued treatment: 4 early deaths, 3 adverse events, and 3 other causes.
  • With a median follow-up of 17 months, 10 patients responded to treatment.
  • All but one of the responders had dexamethasone added to their treatment program.
  • [MeSH-major] Amyloidosis / drug therapy. Amyloidosis / immunology. Dexamethasone / administration & dosage. Immunoglobulin Light Chains / immunology. Thalidomide / analogs & derivatives
  • [MeSH-minor] Administration, Oral. Adult. Aged. Aged, 80 and over. Disease Progression. Dose-Response Relationship, Drug. Drug Administration Schedule. Drug Therapy, Combination. Female. Follow-Up Studies. Humans. Male. Middle Aged. Predictive Value of Tests. Survival Rate. Treatment Outcome

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  • (PMID = 17008538.001).
  • [ISSN] 0006-4971
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA 91561
  • [Publication-type] Clinical Trial; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunoglobulin Light Chains; 4Z8R6ORS6L / Thalidomide; 7S5I7G3JQL / Dexamethasone; F0P408N6V4 / lenalidomide
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28. Messiaen T, Deret S, Mougenot B, Bridoux F, Dequiedt P, Dion JJ, Makdassi R, Meeus F, Pourrat J, Touchard G, Vanhille P, Zaoui P, Aucouturier P, Ronco PM: Adult Fanconi syndrome secondary to light chain gammopathy. Clinicopathologic heterogeneity and unusual features in 11 patients. Medicine (Baltimore); 2000 May;79(3):135-54
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  • The pioneering work by Maldonado and associates (35), who reviewed the first 17 cases in 1975, led to the unifying concept that patients with FS and Bence Jones proteinuria have a special form of plasma cell dyscrasia characterized by slow progression of the tumor and by prominent crystal formation in proximal tubule cells, in the absence of myeloma casts in the distal tubule.
  • Moreover, 10 of the kappa light chains could be entirely or partially sequenced and tested for their resistance to cathepsin B, a lysosomal protease present in proximal tubule cells.
  • One patient of this group also had numerous crystals in proximal tubule cells.
  • The eleventh patient had complete FS with MGUS, but no crystals in proximal tubule cells even after electron microscopy.
  • First, they all were of the kappa type.
  • Resistance of V kappa to proteolysis in FS patients can explain the accumulation of the light chain in the endocytotic compartment of the proximal tubule cells, leading to impairment of proximal tubule functions.
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Female. Humans. Immunoglobulin Light Chains / chemistry. Immunoglobulin Light Chains / urine. Immunoglobulin kappa-Chains / chemistry. Immunoglobulin kappa-Chains / urine. Kidney Tubules, Proximal / pathology. Male. Middle Aged. Multiple Myeloma / etiology. Multiple Myeloma / immunology

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  • (PMID = 10844934.001).
  • [ISSN] 0025-7974
  • [Journal-full-title] Medicine
  • [ISO-abbreviation] Medicine (Baltimore)
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / Immunoglobulin Light Chains; 0 / Immunoglobulin kappa-Chains
  • [Number-of-references] 66
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29. Paciocco G, Bossone E, Erba H, Rubenfire M: Reversible pulmonary hypertension in POEMS syndrome--another etiology of triggered pulmonary vasculopathy? Can J Cardiol; 2000 Aug;16(8):1007-12
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome, a plasma cell dyscrasia associated with pulmonary hypertension, has been treated in the past with anticytokine strategies with a poor outcome.
  • [MeSH-minor] Adrenal Cortex Hormones / administration & dosage. Diuretics / administration & dosage. Drug Therapy, Combination. Female. Humans. Middle Aged. Nifedipine / administration & dosage. Raynaud Disease / complications. Raynaud Disease / diagnosis. Raynaud Disease / drug therapy. Remission Induction. Time Factors


30. Nasr Ben Ammar C, Ghorbel I, Kochbati L, Gargouri W, Touati S, Maalej M: [Solitary and extramedullary plasmocytoma in the head and neck region: five cases report]. Cancer Radiother; 2010 Dec;14(8):755-8
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  • [Transliterated title] Plasmocytome solitaire extramédullaire de la tête et du cou: à propos de cinq cas.
  • All patients received irradiation of 40 to 45 Gy in the primary site associated to surgery in four cases.
  • RESULTS: Four complete responses were noted with a follow-up of 12, 36, 52 and 72 months.
  • Multiple myeloma occurred in one patient 8 years after treatment.
  • CONCLUSION: Radiotherapy is the best effective local treatment.
  • Local control of extramedullary plasmocytoma in the head and neck region seems to be improved when the dose is at least 45 Gy.
  • [MeSH-major] Head and Neck Neoplasms / radiotherapy. Plasmacytoma / radiotherapy
  • [MeSH-minor] Adult. Aged. Antineoplastic Agents / therapeutic use. Combined Modality Therapy. Disease Progression. Ethmoid Bone / pathology. Ethmoid Bone / surgery. Female. Humans. Male. Maxillary Neoplasms / pathology. Maxillary Neoplasms / radiotherapy. Maxillary Neoplasms / surgery. Middle Aged. Multiple Myeloma / drug therapy. Multiple Myeloma / radiotherapy. Neoplasm Invasiveness. Neoplasm Recurrence, Local / radiotherapy. Nose Neoplasms / radiotherapy. Nose Neoplasms / surgery. Remission Induction. Skull Neoplasms / pathology. Skull Neoplasms / radiotherapy. Skull Neoplasms / surgery. Treatment Outcome

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  • [Copyright] Copyright © 2010 Société française de radiothérapie oncologique (SFRO). Published by Elsevier SAS. All rights reserved.
  • (PMID = 20673736.001).
  • [ISSN] 1769-6658
  • [Journal-full-title] Cancer radiothérapie : journal de la Société française de radiothérapie oncologique
  • [ISO-abbreviation] Cancer Radiother
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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31. Mak YK, Chan CH, Chen YT, Lau SM, So CC, Wong KF: Consolidation therapy with autologous blood stem cell transplantation in a patient with primary plasma cell leukaemia. Clin Lab Haematol; 2003 Feb;25(1):55-8
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  • [Title] Consolidation therapy with autologous blood stem cell transplantation in a patient with primary plasma cell leukaemia.
  • Primary plasma cell leukaemia (PPCL) is a rare form of plasma cell dyscrasia.
  • Conventional melphalan-based treatment is often ineffective, with a reported median survival of 2-7 months only.
  • We report a 53-year-old man with PPCL who was treated with four cycles of combination chemotherapy including vincristine, adriamycin and dexamethasone that resulted in a good partial remission.
  • High-dose melphalan 200 mg/m2 and autologous peripheral blood stem cell (PBSC) rescue was then given 6 months after diagnosis.
  • Maintenance interferon-alpha was started 8 weeks after transplantation with good drug compliance.
  • In conclusion, high-dose therapy followed by autologous stem cell rescue is a feasible option for PPCL that can result in a reasonably sustained remission.
  • [MeSH-major] Leukemia, Plasma Cell / therapy. Peripheral Blood Stem Cell Transplantation / methods
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Humans. Interferon-alpha / therapeutic use. Male. Middle Aged. Remission Induction / methods. Transplantation, Autologous

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  • (PMID = 12542443.001).
  • [ISSN] 0141-9854
  • [Journal-full-title] Clinical and laboratory haematology
  • [ISO-abbreviation] Clin Lab Haematol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Interferon-alpha
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32. Raanani P, Gafter-Gvili A, Paul M, Ben-Bassat I, Leibovici L, Shpilberg O: Immunoglobulin prophylaxis in chronic lymphocytic leukemia and multiple myeloma: systematic review and meta-analysis. Leuk Lymphoma; 2009 May;50(5):764-72
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  • The role of intravenous immunoglobulins (IVIG) prophylaxis in hypogammaglobulinemic patients with lymphoproliferative disorders (LPD) and plasma cell dyscrasias (PCD) has not been established.
  • Adverse events, usually not requiring discontinuation of IVIG, occurred significantly more with IVIG.
  • [MeSH-major] Immunoglobulins, Intravenous / therapeutic use. Infection Control / methods. Leukemia, Lymphocytic, Chronic, B-Cell / drug therapy. Multiple Myeloma / drug therapy
  • [MeSH-minor] Immunologic Factors / therapeutic use. Infection / drug therapy. Premedication. Randomized Controlled Trials as Topic. Survival Rate. Treatment Outcome

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  • (PMID = 19330654.001).
  • [ISSN] 1029-2403
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Publication-type] Journal Article; Meta-Analysis; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Immunoglobulins, Intravenous; 0 / Immunologic Factors
  • [Number-of-references] 20
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33. Dispenzieri A: POEMS syndrome. Blood Rev; 2007 Nov;21(6):285-99
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • POEMS syndrome is a rare paraneoplastic syndrome secondary to a plasma cell dyscrasia.
  • Recognition of the complex of a combination of peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasmaproliferative disorder, skin changes, papilledema, extravascular volume overload (peripheral edema, pleural effusions, ascites), sclerotic bone lesions, thrombocytosis, Castleman disease is the first step in effectively managing the disease.
  • In patients with a dominant sclerotic plasmacytoma, first line therapy should include radiation to the lesion.
  • Retrospective analysis and personal experience would dictate that systemic therapy be considered for patients with diffuse sclerotic lesions or absence of any bone lesion and for those who have not demonstrated stabilization of their disease 3 to 6 months after completing radiation therapy.
  • For those patients with diffuse disease, systemic therapy is indicated.
  • Useful approaches include therapy with corticosteroids, low dose alkylator therapy, and high dose chemotherapy with peripheral blood stem cell transplant.
  • Until the pathogenesis is fully understood, these are the mainstays of treatment for patients with POEMS syndrome.
  • The role of anti-VEGF therapies, immune modulatory drugs, and proteasome inhibitors has not yet been defined, but drugs with known high rates of treatment related neuropathy should not be considered as first line therapy.
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Chemotherapy, Adjuvant / methods. Giant Lymph Node Hyperplasia / complications. Hematopoietic Stem Cell Transplantation / methods. Humans. Respiratory Function Tests

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  • (PMID = 17850941.001).
  • [ISSN] 0268-960X
  • [Journal-full-title] Blood reviews
  • [ISO-abbreviation] Blood Rev.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Scotland
  • [Chemical-registry-number] 0 / VEGFA protein, human; 0 / Vascular Endothelial Growth Factor A
  • [Number-of-references] 165
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34. Sezer O, Eucker J, Jakob C, Possinger K: Diagnosis and treatment of AL amyloidosis. Clin Nephrol; 2000 Jun;53(6):417-23
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Diagnosis and treatment of AL amyloidosis.
  • AL (amyloid light-chain) amyloidosis is a plasma cell disorder in which depositions of amyloid light-chain protein cause progressive organ failure.
  • Virtually all patients with AL amyloidosis have a monoclonal protein in the serum or urine or a monoclonal population of plasma cells in the bone marrow.
  • It is important to recognize that the amyloidosis is a dynamic process, and chemotherapy induced reduction of the activity of the plasma cell clone reduces the supply of the amyloid precursor protein and can result in a major regression of the deposits.
  • Conventional-dose melphalan as standard treatment can prolong the median duration of survival about 10 months, but the clinical response rates with improvement of impaired organ function are low with a slow response.
  • Upfront high-dose chemotherapy with autologous peripheral blood stem cell transplantation is much more effective and can result in a major improvement of the patient's clinical condition, but the treatment-related toxicity can be relevant due to impaired organ function.
  • The initial use of a conventional-dose chemotherapy consisting of vincristine, doxorubicin and dexamethasone (VAD) to achieve a remission and subsequent high-dose chemotherapy is the concept of a German trial.
  • The improvement of the condition of the patient by this approach may increase the tolerability of high-dose chemotherapy and reduce transplantation-related problems.
  • [MeSH-major] Amyloid / chemistry. Amyloidosis / diagnosis. Amyloidosis / therapy. Nephrotic Syndrome / etiology. Proteinuria / etiology
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Doxorubicin / analogs & derivatives. Doxorubicin / therapeutic use. Hematopoietic Stem Cell Transplantation. Humans. Prognosis

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  • [CommentIn] Clin Nephrol. 2001 Jan;55(1):86 [11200875.001]
  • (PMID = 10879660.001).
  • [ISSN] 0301-0430
  • [Journal-full-title] Clinical nephrology
  • [ISO-abbreviation] Clin. Nephrol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] GERMANY
  • [Chemical-registry-number] 0 / Amyloid; 0 / Antineoplastic Agents; 80168379AG / Doxorubicin; 83997-75-5 / 4'-deoxy-4'-iododoxorubicin
  • [Number-of-references] 38
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35. Hussein MA: Thalidomide: present and future in multiple myeloma. Expert Rev Anticancer Ther; 2005 Feb;5(1):25-31
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Multiple myeloma continues to be an incurable disease.
  • The understanding of the disease's pathophysiology has significantly improved over the past few years, partly due to the discovery of the role of immunomodulatory agents and the study of their mechanism of action.
  • Thalidomide, the first of the immunomodulatory family to be used in the management of multiple myeloma, proved not only to be effective in the treatment of multiple myeloma, but also instigated a wide range of in vitro and in vivo studies to define the pathophysiology of the plasma cell dyscrasia.
  • The attention thalidomide has received in the past and recent history has not been without a price.
  • The drug has a side-effect profile that, if managed appropriately, provides the most unique active molecule in the management of the disease, where it maintains the same response rate in newly diagnosed patients as in advanced relapsed/refractory multiple myeloma patients.
  • [MeSH-major] Angiogenesis Inhibitors / therapeutic use. Multiple Myeloma / drug therapy. Thalidomide / therapeutic use
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Clinical Trials as Topic. Humans. Prognosis. Recurrence

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  • (PMID = 15757435.001).
  • [ISSN] 1744-8328
  • [Journal-full-title] Expert review of anticancer therapy
  • [ISO-abbreviation] Expert Rev Anticancer Ther
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 4Z8R6ORS6L / Thalidomide
  • [Number-of-references] 58
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36. Cheung WY, Greenberg CR, Bernstein K, Schacter B, Fourie T, Seftel MD: Type I Gaucher disease following chemotherapy for light chain multiple myeloma. Intern Med; 2007;46(15):1255-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Type I Gaucher disease following chemotherapy for light chain multiple myeloma.
  • Although plasma cell disorders, such as hypergammaglobulinemia and monoclonal gammopathy of undetermined significance (MGUS), are reported to occur at higher incidences in patients with Type I Gaucher disease (GD) than in the normal population, pure light chain multiple myeloma (LCMM) has never been described in this context.
  • Our case is the first to highlight a patient with LCMM who developed clinically apparent GD only following chemotherapy and hematopoietic stem cell transplantation.
  • [MeSH-major] Gaucher Disease / chemically induced. Hematopoietic Stem Cell Transplantation / adverse effects. Multiple Myeloma / drug therapy
  • [MeSH-minor] Antineoplastic Agents / adverse effects. Antineoplastic Agents / therapeutic use. Female. Humans. Middle Aged. Nephrotic Syndrome / diagnosis. Nephrotic Syndrome / etiology


37. Zenhäusern R, Tobler A, Leoncini L, Hess OM, Ferrari P: Fatal cardiac arrhythmia after infusion of dimethyl sulfoxide-cryopreserved hematopoietic stem cells in a patient with severe primary cardiac amyloidosis and end-stage renal failure. Ann Hematol; 2000 Sep;79(9):523-6
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  • Amyloidosis (AL) is a rapidly fatal plasma cell dyscrasia causing progressive multiorgan failure.
  • Recently, substantial improvement of survival was reported following high-dose chemotherapy with peripheral blood stem cell (PBSC) rescue.
  • Immediately after the second infusion of the PBSCs, life-threatening cardiac arrhythmias occurred and, despite intensive treatment, the patient died less than 24 h later.
  • Given the poor prognosis of AL and the promising results of dose-intensive chemotherapy with autologous PBSC transplantation, careful patient selection and intensive monitoring are mandatory in order to further pursue this therapeutic approach.
  • [MeSH-major] Amyloidosis / therapy. Arrhythmias, Cardiac / chemically induced. Cardiomyopathies / therapy. Cryopreservation. Dimethyl Sulfoxide / adverse effects. Kidney Failure, Chronic / therapy. Stem Cells
  • [MeSH-minor] Fatal Outcome. Heart Arrest / etiology. Hematopoietic Stem Cell Transplantation. Humans. Male. Middle Aged


38. Kim SJ, Kim J, Cho Y, Seo BK, Kim BS: Combination chemotherapy with bortezomib, cyclophosphamide and dexamethasone may be effective for plasma cell leukemia. Jpn J Clin Oncol; 2007 May;37(5):382-4
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  • [Title] Combination chemotherapy with bortezomib, cyclophosphamide and dexamethasone may be effective for plasma cell leukemia.
  • Plasma cell leukemia is a rare malignant plasma cell disorder characterized by proliferation of plasma cells in blood and the bone marrow, the outcome of which is poor with conventional therapy.
  • More effective treatment strategies are therefore needed for this disorder.
  • Here, we report a case of secondary plasma cell leukemia from Immunoglobulin D multiple myeloma refractory to doxorubicin-containing chemotherapy and thalidomide.
  • The patient achieved complete remission with bortezomib-containing chemotherapy as follows: bortezomib 1.3 mg/m2 intravenous infusion on days 1, 4, 8 and 11; cyclophosphamide 750 mg/m(2) intravenous infusion on days 1 and 3; dexamethasone 40 mg/m2 intravenous infusion on days 1-4.
  • Complete remission was maintained until the fourth course of the treatment, and we then performed autologous peripheral blood stem cell transplantation.
  • Our experience suggests that combination chemotherapy with bortezomib, cyclophosphamide and dexamethasone may be an effective induction treatment for plasma cell leukemia.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Leukemia, Plasma Cell / drug therapy

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  • (PMID = 17538191.001).
  • [ISSN] 1465-3621
  • [Journal-full-title] Japanese journal of clinical oncology
  • [ISO-abbreviation] Jpn. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Antineoplastic Agents, Alkylating; 0 / Antineoplastic Agents, Hormonal; 0 / Boronic Acids; 0 / Pyrazines; 69G8BD63PP / Bortezomib; 7S5I7G3JQL / Dexamethasone; 8N3DW7272P / Cyclophosphamide
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39. Koike H, Sobue G: Crow-Fukase syndrome. Neuropathology; 2000 Sep;20 Suppl:S69-72
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Crow-Fukase syndrome is a unique multisystem disorder that is also known as POEMS syndrome (an acronym for polyneuropathy, organomegaly, endocrinopathy, the presence of M-protein and skin change).
  • This syndrome is strongly associated with plasma cell dyscrasia.
  • M-protein, interleukin (IL)-1beta, IL-6 and tumor necrosis factor (TNF)-alpha are also considered to be involved in the pathogenesis.
  • Treatment consists of radiation and surgical resection of the myeloma, chemotherapy, and a high dose of intravenous immunoglobulin (IVIg).

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  • (PMID = 11037192.001).
  • [ISSN] 0919-6544
  • [Journal-full-title] Neuropathology : official journal of the Japanese Society of Neuropathology
  • [ISO-abbreviation] Neuropathology
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] AUSTRALIA
  • [Number-of-references] 21
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40. Raj RS, Najeeb S, Aruna R, Pavithran K, Thomas M: Primary plasma cell leukemia occuring in the young. Indian J Cancer; 2003 Jul-Sep;40(3):116-7
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  • [Title] Primary plasma cell leukemia occuring in the young.
  • Plasma Cell Leukemia (PCL) is a rare form of plasma cell dyscrasia.
  • Plasma cell leukemia has two variants: the primary form presents de novo in patients with no previous history of multiple myeloma (MM); the secondary form consists of a leukemic transformation in a previously recognized MM.
  • She was treated with combination chemotherapy (VAD).
  • Although she had a good response initially, later the disease progressed and she died 6 months after the diagnosis.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Dexamethasone / therapeutic use. Doxorubicin / therapeutic use. Leukemia, Plasma Cell / drug therapy. Vincristine / therapeutic use
  • [MeSH-minor] Adult. Disease Progression. Fatal Outcome. Female. Humans. Prognosis

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  • (PMID = 14716116.001).
  • [ISSN] 0019-509X
  • [Journal-full-title] Indian journal of cancer
  • [ISO-abbreviation] Indian J Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] India
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 7S5I7G3JQL / Dexamethasone; 80168379AG / Doxorubicin; VAD protocol
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41. Ishigaki T, Sasaki K, Watanabe K, Nakamura N, Toyota S, Kobayashi H, Tohda S: Amplification of IGH/CCND1 fusion gene in a primary plasma cell leukemia case. Cancer Genet Cytogenet; 2010 Aug;201(1):62-5
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  • [Title] Amplification of IGH/CCND1 fusion gene in a primary plasma cell leukemia case.
  • The IGH/CCND1 fusion gene has been reported in many hematologic tumors such as mantle cell lymphoma, chronic lymphocytic leukemia, prolymphocytic leukemia, multiple myeloma, and plasma cell leukemia.
  • We report a case of plasma cell leukemia showing five IGH/CCND1 fusion signals by interphase fluorescence in situ hybridization (FISH).
  • Conventional cytogenetic analysis and multicolor spectral karyotyping showed a complex karyotype that did not include t(11;14).
  • The patient was treated with intensive chemotherapy.
  • The amplification of the IGH/CCND1 fusion gene may contribute to the aggressive course of the disease.
  • To our knowledge, this is the first case showing amplification of the IGH/CCND1 gene in plasma cell neoplasms.
  • [MeSH-major] Cyclin D1 / genetics. Immunoglobulin Heavy Chains / genetics. Leukemia, Plasma Cell / genetics

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  • [Copyright] Copyright (c) 2010 Elsevier Inc. All rights reserved.
  • (PMID = 20633772.001).
  • [ISSN] 1873-4456
  • [Journal-full-title] Cancer genetics and cytogenetics
  • [ISO-abbreviation] Cancer Genet. Cytogenet.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / CCND1 protein, human; 0 / Immunoglobulin Heavy Chains; 136601-57-5 / Cyclin D1
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42. Kovacsovics T: [Current treatment of AL amyloidosis]. Rev Med Suisse Romande; 2000 Oct;120(10):771-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Current treatment of AL amyloidosis].
  • [Transliterated title] Approches actuelles du traitement de l'amyloïdose AL.
  • AL amyloidosis is a plasma cell disorder characterized by the tissue accumulation of immunoglobulin light chains.
  • The prognosis of AL amyloidosis is poor, with a median survival inferior to two years.
  • Amyloid deposits, responsible for the clinical manifestations of this disease, can regress at least partially after the suppression of the production of amyloid precursors.
  • Therefore, current treatment strategies in AL amyloidosis aim at reducing the plasma cell clones, using chemotherapy regimens applied in multiple myeloma.
  • A combination of melphalan and prednisone is the standard therapy of AL amyloidosis, but its results remain disappointing.
  • Intensive chemotherapy regimens, with high-dose melphalan followed by peripheral blood stem cell transplantation, lead to increased response rates and improved survival, but are complicated by severe short term morbidity and mortality.
  • [MeSH-major] Amyloid. Amyloidosis / classification. Amyloidosis / therapy. Anti-Inflammatory Agents / therapeutic use. Antineoplastic Agents, Alkylating / therapeutic use. Hematopoietic Stem Cell Transplantation. Melphalan / therapeutic use. Prednisone / therapeutic use
  • [MeSH-minor] Combined Modality Therapy. Humans. Morbidity. Prognosis. Remission Induction. Survival Analysis. Treatment Outcome

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  • (PMID = 11109905.001).
  • [ISSN] 0035-3655
  • [Journal-full-title] Revue médicale de la Suisse romande
  • [ISO-abbreviation] Rev Med Suisse Romande
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] SWITZERLAND
  • [Chemical-registry-number] 0 / Amyloid; 0 / Anti-Inflammatory Agents; 0 / Antineoplastic Agents, Alkylating; 0 / amyloid protein AL; Q41OR9510P / Melphalan; VB0R961HZT / Prednisone
  • [Number-of-references] 25
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43. Arat M, Ulusoy V, Demirer T, Uysal AV, Ozcan M, Dinçer S, Ilhan O, Koç H: An unusual presentation of plasma cell dyscrasias: cardiac tamponade due to myelomatous infiltration. Leuk Lymphoma; 2002 Jan;43(1):145-8
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  • [Title] An unusual presentation of plasma cell dyscrasias: cardiac tamponade due to myelomatous infiltration.
  • Pericardial involvement, a rare complication of multiple myeloma (MM), is caused by amyloidosis, infections, bleeding abnormalities or plasma cell infiltration, usually at a late or terminal stage of the disease.
  • The pericardiocentesis fluid showed infiltration with plasma cells in one of the three patients, who had a progressive and fatal course.
  • In the second patient pericardial invasion was proven by biopsy and the third was diagnosed with a plasma cell leukemia but developed a pericardial effusion demonstrated by pericardial biopsy.
  • All these three patients died of progressive disease without any response to chemotherapy and supportive measures.
  • In conclusion, optimal treatment for malignant involvement of the pericardium by myeloma cells has not yet been established and is often fatal.
  • [MeSH-minor] Adult. Aged. Female. Heart Neoplasms / complications. Heart Neoplasms / pathology. Humans. Male. Middle Aged. Neoplasm Invasiveness. Paraproteinemias / complications. Paraproteinemias / pathology. Pericardial Effusion / complications. Pericardial Effusion / etiology. Pericarditis / etiology. Pericarditis / pathology

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  • (PMID = 11908719.001).
  • [ISSN] 1042-8194
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
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44. Schonland SO, Perz JB, Hundemer M, Hegenbart U, Kristen AV, Hund E, Dengler TJ, Beimler J, Zeier M, Singer R, Linke RP, Ho AD, Goldschmidt H: Indications for high-dose chemotherapy with autologous stem cell support in patients with systemic amyloid light chain amyloidosis. Transplantation; 2005 Sep 27;80(1 Suppl):S160-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Indications for high-dose chemotherapy with autologous stem cell support in patients with systemic amyloid light chain amyloidosis.
  • Systemic amyloid light chain amyloidosis is a protein conformation disorder caused by a clonal plasma cell dyscrasia.
  • Symptoms result from fibrillar extracellular deposits in kidney, heart, liver, gut, peripheral nervous system and other tissues.
  • Using conventional chemotherapy, the median survival could be prolonged by 4 months.
  • Treatment with high-dose melphalm (HDM) and autologous stem cell transplantation (ASCT) of selected patients has been shown to arrest and even to reverse the disease course.
  • This procedure however remains controversial because treatment related mortality (TRM) in AL amyloidosis is substantially higher (15-40%) than in multiple myeloma (<5%).
  • Here we review recent results of ASCT, eligibility criteria for HDM and report our own treatment results in 41 patients.
  • [MeSH-major] Amyloidosis / therapy. Immunoglobulin Light Chains. Melphalan / therapeutic use. Stem Cell Transplantation
  • [MeSH-minor] Adult. Aged. Child, Preschool. Clinical Trials as Topic. Combined Modality Therapy. Female. Humans. Male. Middle Aged. Retrospective Studies. Transplantation, Autologous

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  • (PMID = 16286897.001).
  • [ISSN] 0041-1337
  • [Journal-full-title] Transplantation
  • [ISO-abbreviation] Transplantation
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunoglobulin Light Chains; Q41OR9510P / Melphalan
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45. Lommatzsch SE, Bellizzi AM, Cathro HP, Rosner MH: Acute renal failure caused by renal infiltration by hematolymphoid malignancy. Ann Diagn Pathol; 2006 Aug;10(4):230-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Renal involvement by a malignant infiltrative process is often suspected in patients with bilaterally enlarged kidneys and concurrent malignancies.
  • We present 2 cases in which ARF is attributable to malignant hematolymphoid infiltration.
  • The patient's renal function improved dramatically after the initiation of chemotherapy, clearly linking the development of ARF to the malignant process.
  • In the second case, infiltration of the kidneys by plasma cell leukemia resulted in dialysis dependence.
  • To our knowledge, this represents the first reported case of ARF attributable to documented renal infiltration by plasma cell leukemia.
  • A review of the potential causes of renal failure in hematolymphoid malignancy, focusing on the direct impact of the infiltrative process and on the spectrum of renal disease in plasma cell dyscrasia, is presented.
  • [MeSH-major] Acute Kidney Injury / etiology. Kidney / pathology. Leukemia, Plasma Cell / complications. Leukemic Infiltration. Lymphoma, Non-Hodgkin / complications
  • [MeSH-minor] Allopurinol / therapeutic use. Antimetabolites / therapeutic use. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Dexamethasone / therapeutic use. Female. Humans. Middle Aged. Thalidomide / therapeutic use. Treatment Outcome

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  • (PMID = 16844565.001).
  • [ISSN] 1092-9134
  • [Journal-full-title] Annals of diagnostic pathology
  • [ISO-abbreviation] Ann Diagn Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antimetabolites; 4Z8R6ORS6L / Thalidomide; 63CZ7GJN5I / Allopurinol; 7S5I7G3JQL / Dexamethasone
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46. Zeier M, Perz J, Linke RP, Donini U, Waldherr R, Andrassy K, Ho AD, Goldschmidt H: No regression of renal AL amyloid in monoclonal gammopathy after successful autologous blood stem cell transplantation and significant clinical improvement. Nephrol Dial Transplant; 2003 Dec;18(12):2644-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] No regression of renal AL amyloid in monoclonal gammopathy after successful autologous blood stem cell transplantation and significant clinical improvement.
  • BACKGROUND: High-dose chemotherapy followed by autologous blood stem cell transplantation induces remission of plasma cell dyscrasia in patients with AL amyloidosis.
  • The impact of this treatment on the glomerular amyloid mass is still unknown.
  • METHODS: In the present study, the quantity of the renal amyloid mass before and more than 3 years after high-dose melphalan treatment and autologous blood stem cell transplantation was assessed in two patients.
  • At the time of the second renal biopsy, both patients were in complete remission without detectable serum and urinary monoclonal IgA-lambda and a normal percentage of plasma cells in the bone marrow.
  • RESULTS: In both patients with biopsy-proven AL amyloidosis, urinary protein excretion decreased from 7 g/24 h to <2 g/24 h more than 3 years after autologous blood stem cell transplantation.
  • CONCLUSION: Despite complete remission of the plasma cell dyscrasia and improvement of glomerular permeability, the amount of glomerular amyloid mass did not regress.
  • [MeSH-major] Amyloid / analysis. Amyloidosis / physiopathology. Antineoplastic Agents, Alkylating / therapeutic use. Hematopoietic Stem Cell Transplantation / methods. Kidney Diseases / physiopathology. Melphalan / therapeutic use. Paraproteinemias / physiopathology

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  • (PMID = 14605290.001).
  • [ISSN] 0931-0509
  • [Journal-full-title] Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association
  • [ISO-abbreviation] Nephrol. Dial. Transplant.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Amyloid; 0 / Antineoplastic Agents, Alkylating; Q41OR9510P / Melphalan
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47. Hogan WJ, Lacy MQ, Wiseman GA, Fealey RD, Dispenzieri A, Gertz MA: Successful treatment of POEMS syndrome with autologous hematopoietic progenitor cell transplantation. Bone Marrow Transplant; 2001 Aug;28(3):305-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Successful treatment of POEMS syndrome with autologous hematopoietic progenitor cell transplantation.
  • POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) is a plasma cell dyscrasia that differs substantially from classic multiple myeloma.
  • Current therapeutic approaches are frequently inadequate and leave many patients wheelchair-bound with significant deterioration in quality and length of life.
  • We present the case of a young man with progressive disease despite conventional therapeutic approaches.
  • We describe a novel approach to treatment with a bone-seeking radiopharmaceutical, samarium-153 ethylene diamine tetramethylene phosphonate ((153)Sm-EDTMP), followed by myeloablative chemotherapy with autologous hematopoietic progenitor cell reconstitution.
  • An aggressive approach should be considered in patients with POEMS syndrome in whom standard therapeutic measures fail.
  • [MeSH-major] Hematopoietic Stem Cell Transplantation. Organometallic Compounds / administration & dosage. Organophosphorus Compounds / administration & dosage. POEMS Syndrome / therapy
  • [MeSH-minor] Adult. Antineoplastic Agents, Alkylating / administration & dosage. Dose-Response Relationship, Radiation. Drug Therapy, Combination. Graft Survival. Humans. Male. Melphalan / administration & dosage. Quality of Life. Radiopharmaceuticals / administration & dosage. Radiopharmaceuticals / pharmacokinetics. Radiopharmaceuticals / therapeutic use. Transplantation, Autologous. Treatment Outcome

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  • [CommentIn] Bone Marrow Transplant. 2002 Jul;30(1):61-2 [12105781.001]
  • (PMID = 11536000.001).
  • [ISSN] 0268-3369
  • [Journal-full-title] Bone marrow transplantation
  • [ISO-abbreviation] Bone Marrow Transplant.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 0 / Organometallic Compounds; 0 / Organophosphorus Compounds; 0 / Radiopharmaceuticals; 122575-21-7 / samarium ethylenediaminetetramethylenephosphonate; Q41OR9510P / Melphalan
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48. Dispenzieri A: POEMS Syndrome. Hematology Am Soc Hematol Educ Program; 2005;:360-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • POEMS syndrome is defined by the presence of a peripheral neuropathy (P), a monoclonal plasma cell disorder (M), and other paraneoplastic features, the most common of which include organomegaly (O), endocrinopathy (E), skin changes (S), papilledema, edema, effusions, ascites, and thrombocytosis.
  • Virtually all patients will have either sclerotic bone lesion(s) or co-existent Castleman's disease.
  • Not all features of the disease are required to make the diagnosis, and early recognition is important to reduce morbidity.
  • Not until additional features of the POEMS syndrome are recognized is the correct diagnosis made and effective therapies initiated.
  • Therapies that may be effective in patients with CIDP and MGUS-associated peripheral neuropathy (intravenous gammaglobulin and plasmapheresis) are not effective in patients with POEMS.
  • Instead, the mainstays of therapy for patients with POEMS include irradiation, corticosteroids, and alkylator-based therapy, including high-dose chemotherapy with peripheral blood stem cell transplantation.

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  • (PMID = 16304404.001).
  • [ISSN] 1520-4383
  • [Journal-full-title] Hematology. American Society of Hematology. Education Program
  • [ISO-abbreviation] Hematology Am Soc Hematol Educ Program
  • [Language] ENG
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Adrenal Cortex Hormones; 0 / gamma-Globulins
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49. Treon SP, Anderson KC: The use of rituximab in the treatment of malignant and nonmalignant plasma cell disorders. Semin Oncol; 2000 Dec;27(6 Suppl 12):79-85
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The use of rituximab in the treatment of malignant and nonmalignant plasma cell disorders.
  • CD20 is a B-cell-restricted antigen that, for the most part, is expressed from the pre-B-cell to the mature B-cell stage of B-cell differentiation.
  • Several transcription factors regulate CD20 expression during B-cell differentiation, the most important of which appear to be PU.1 and Pip (PU.1 interacting protein).
  • As B cells differentiate to plasma cells, CD20 expression is down-regulated, which coincides with PU.1 downregulation in plasma cells.
  • Analogous to their normal B-cell counterparts, CD20 is expressed on malignant lymphoplasmacytic cells from most patients with Waldenstrom's macroglobulinemia and on malignant plasma cells from a fraction (20%) of multiple myeloma patients.
  • CD20 also is expressed on subpopulations of normal donor plasma cells, which may include autoantibody-secreting plasmacytes.
  • In view of these findings, the anti-CD20 chimeric monoclonal antibody, rituximab (Rituxan; Genentech, Inc, South San Francisco, CA and IDEC Pharmaceutical Corporation, San Diego, CA), has been evaluated in the treatment of Waldenstrom's macroglobulinemia and multiple myeloma, as well as in nonmalignant plasma cell disorders including IgM polyneuropathies, immune thrombocytopenias, and autoimmune hemolytic anemias, with reported activity in these entities.
  • [MeSH-major] Antibodies, Monoclonal / therapeutic use. Antineoplastic Agents / therapeutic use. Paraproteinemias / drug therapy
  • [MeSH-minor] Antibodies, Monoclonal, Murine-Derived. Antigens, CD20 / genetics. B-Lymphocytes / physiology. Gene Expression Regulation / drug effects. Humans. Immunoglobulin M / immunology. Multiple Myeloma / drug therapy. Purpura, Thrombocytopenic, Idiopathic / drug therapy. Rituximab. Transcription Factors. Waldenstrom Macroglobulinemia / drug therapy

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  • (PMID = 11226004.001).
  • [ISSN] 0093-7754
  • [Journal-full-title] Seminars in oncology
  • [ISO-abbreviation] Semin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Murine-Derived; 0 / Antigens, CD20; 0 / Antineoplastic Agents; 0 / Immunoglobulin M; 0 / Transcription Factors; 4F4X42SYQ6 / Rituximab
  • [Number-of-references] 49
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50. Ansari-Lari MA, Ali SZ: Fine-needle aspiration of abdominal fat pad for amyloid detection: a clinically useful test? Diagn Cytopathol; 2004 Mar;30(3):178-81
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  • [Title] Fine-needle aspiration of abdominal fat pad for amyloid detection: a clinically useful test?
  • Fine-needle aspiration of abdominal fat pad (FNAFP) is commonly employed for the diagnosis of systemic amyloidosis, a disease with highly variable clinical manifestations, often presenting difficult patient management problems.
  • Major emphases were assessment of the clinical utility of the test, correlation with concurrent or subsequent biopsies, and treatment strategies.
  • The primary indications for FNAFP were monoclonal gammopathy (34%), cardiomyopathy (22%), renal insufficiency (20%), neuropathy (8%), plasma cell dyscrasia (6%), and other conditions (10%).
  • Twenty-one patients positive for amyloid, based on initial or follow-up biopsies, were managed symptomatically without any specific treatment for amyloidosis.
  • One patient, who was specifically treated for amyloidosis by melphalan and dexamethasone, died 1 wk after therapy.
  • Three patients with multiple myeloma and amyloidosis underwent chemotherapy.
  • An FNAFP result is often not considered clinically conclusive and is followed by further invasive procedures to detect amyloid (55% of our positive and 31% of our negative FNAFP cases were rebiopsied).
  • Although in the majority of cases diagnosis of amyloidosis did not alter the treatment strategies, a conclusive positive result helped in ruling out other underlying conditions as the cause of patients' symptoms.
  • [MeSH-major] Adipose Tissue / pathology. Amyloid / metabolism. Amyloidosis / diagnosis. Biopsy, Fine-Needle / methods

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  • [Copyright] Copyright 2004 Wiley-Liss, Inc.
  • (PMID = 14986298.001).
  • [ISSN] 8755-1039
  • [Journal-full-title] Diagnostic cytopathology
  • [ISO-abbreviation] Diagn. Cytopathol.
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Amyloid; 0 / Coloring Agents; 3U05FHG59S / Congo Red
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51. Bogen B: A mouse model for immunotherapy of myeloma. Hematol J; 2002;3(5):224-9
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  • The variable (V) regions of myeloma proteins are unique to each plasma cell tumor, and therefore contain highly tumor-specific antigenic determinants called idiotopes (Id).
  • We have developed a mouse model to study the molecular and cellular mechanisms for how Id-specific T cell protect against myeloma.
  • A T cell receptor (TCR) transgenic mouse model has been established.
  • However, once a tumor is established, Id-specific T cells become incapacitated.
  • Based on these results, it is suggested that Id-vaccination in humans should be reserved for eradication of minimal residual disease, eg after high dose chemotherapy and stem cell transplantation.

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  • (PMID = 12391539.001).
  • [ISSN] 1466-4860
  • [Journal-full-title] The hematology journal : the official journal of the European Haematology Association
  • [ISO-abbreviation] Hematol. J.
  • [Language] ENG
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, Neoplasm; 0 / Histocompatibility Antigens Class II; 0 / Immunoglobulin Idiotypes
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52. Kelly JJ: Neurologic complications of primary systemic amyloidosis. Rev Neurol Dis; 2006;3(4):173-81
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  • The deposits in primary systemic amyloidosis are derived from monoclonal serum proteins in plasma cell dyscrasia, which are degraded locally in tissues and deposited in insoluble sheets that damage organs.
  • Symptoms of the disorder include neuropathy, myopathy, and cardiac or renal insufficiency; there is often multiple-organ involvement.
  • Differential difficulties exist in diagnosing the disorder, and familial amyloid polyneuropathy directly mimics the disease.
  • Diagnostic tools include electromyography, laboratory testing for abnormalities in serum and urine, and histological investigation of appropriate tissue.
  • However, melphalan and prednisone treatment for at least 1 year has resulted in increased survival rates.
  • There have also been reports of benefit from high-dose chemotherapy followed by peripheral blood stem cell transplantation.
  • Without early therapy, however, the disease has a dismal prognosis, and peripheral neuropathy usually persists or worsens despite therapy and improvement in other organs.

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  • (PMID = 17224900.001).
  • [ISSN] 1545-2913
  • [Journal-full-title] Reviews in neurological diseases
  • [ISO-abbreviation] Rev Neurol Dis
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 57
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53. Gertz MA, Rajkumar SV: Primary systemic amyloidosis. Curr Treat Options Oncol; 2002 Jun;3(3):261-71
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  • Primary amyloidosis is a plasma cell dyscrasia in which insoluble immunoglobulin light chain fragments are produced and polymerize into fibrils that deposit extracellularly, causing visceral organ dysfunction and death.
  • The disorder is rare.
  • The least invasive source of tissue for amyloid detection is the subcutaneous fat.
  • Therapies used include oral melphalan/prednisone and high-dose corticosteroids.
  • High-dose chemotherapy followed by stem cell reconstitution seems to provide the highest reported response rates.
  • Transplant is associated with unique morbidities not seen in the transplantation of patients with other hematologic malignancies.
  • [MeSH-major] Amyloidosis / therapy
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Heart Transplantation. Hematopoietic Stem Cell Transplantation. Humans. Melphalan / therapeutic use. Prednisone / therapeutic use. Prognosis. Survival Rate

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  • (PMID = 12057072.001).
  • [ISSN] 1527-2729
  • [Journal-full-title] Current treatment options in oncology
  • [ISO-abbreviation] Curr Treat Options Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] Q41OR9510P / Melphalan; VB0R961HZT / Prednisone
  • [Number-of-references] 50
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54. Bogen B, Schenck K, Munthe LA, Dembic Z: Deletion of idiotype (Id)-specific T cells in multiple myeloma. Acta Oncol; 2000;39(7):783-8
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  • The variable (V) regions of myeloma proteins are unique to each plasma cell tumor, and therefore contain highly tumor-specific antigenic determinants called idiotopes (Id).
  • We suggest that Id-vaccination should be reserved for eradication of minimal residual disease, e.g. after high-dose chemotherapy and stem-cell transplantation.
  • [MeSH-minor] Animals. Cell Death. Cell Differentiation. Disease Progression. Hematopoietic Stem Cell Transplantation. Humans. Mice. Mice, Transgenic. Neoplasm, Residual. Vaccination

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  • (PMID = 11145433.001).
  • [ISSN] 0284-186X
  • [Journal-full-title] Acta oncologica (Stockholm, Sweden)
  • [ISO-abbreviation] Acta Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Norway
  • [Chemical-registry-number] 0 / Immunoglobulin Idiotypes; 0 / Myeloma Proteins
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55. Gertz MA, Lacy MQ, Dispenzieri A, Gastineau DA, Chen MG, Ansell SM, Inwards DJ, Micallef IN, Tefferi A, Litzow MR: Stem cell transplantation for the management of primary systemic amyloidosis. Am J Med; 2002 Nov;113(7):549-55
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  • [Title] Stem cell transplantation for the management of primary systemic amyloidosis.
  • PURPOSE: To review the characteristics and outcomes of amyloidosis patients treated with high-dose chemotherapy and stem cell reconstitution.
  • All patients had evidence of a clonal plasma cell dyscrasia; those with nonimmunoglobulin forms of amyloidosis were excluded, as were those who had no symptoms of amyloidosis, purpura, carpal tunnel syndrome, or symptomatic multiple myeloma.
  • All patients received melphalan-based chemotherapy; 17 patients were conditioned with total body irradiation.
  • Treatment-related mortality for stem cell transplantation was 14% (9/66).
  • CONCLUSION: The number of organs involved before stem cell transplantation for amyloidosis is the most important factor in predicting subsequent survival.
  • Stem cell transplantation should be considered as a treatment option for selected patients with amyloidosis.
  • [MeSH-major] Amyloidosis / surgery. Stem Cell Transplantation
  • [MeSH-minor] Adult. Aged. Amyloid Neuropathies / surgery. Biomarkers / analysis. Biopsy. Cardiomyopathies / surgery. Female. Humans. Kidney Diseases / surgery. Liver Diseases / surgery. Male. Middle Aged. Peripheral Nerves / metabolism. Retrospective Studies. Survival Analysis. Transplantation Conditioning. Treatment Outcome. Whole-Body Irradiation

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  • (PMID = 12459400.001).
  • [ISSN] 0002-9343
  • [Journal-full-title] The American journal of medicine
  • [ISO-abbreviation] Am. J. Med.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers
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56. Kuwabara S, Misawa S, Kanai K, Sawai S, Hattori T, Nishimura M, Nakaseko C: Thalidomide reduces serum VEGF levels and improves peripheral neuropathy in POEMS syndrome. J Neurol Neurosurg Psychiatry; 2008 Nov;79(11):1255-7
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  • BACKGROUND: Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS) syndrome is a rare multi-system disorder associated with plasma-cell dyscrasia.
  • Several case series and reports have suggested that high-dose chemotherapy with autologous peripheral blood stem-cell transplantation is efficacious treatment, but this transplantation is not indicated for elderly patients and patients with renal failure.
  • OBJECTIVE: To investigate the effects of thalidomide treatment for POEMS syndrome.
  • METHODS: Nine patients, who were not indicated for high-dose chemotherapy, were treated with thalidomide.
  • CONCLUSION: Thalidomide treatment should be further studied as a treatment for POEMS syndrome, particularly for patients who are not indicated for transplantation therapy.
  • [MeSH-major] Angiogenesis Inhibitors / pharmacology. Angiogenesis Inhibitors / therapeutic use. POEMS Syndrome / blood. POEMS Syndrome / drug therapy. Peripheral Nervous System Diseases / drug therapy. Peripheral Nervous System Diseases / physiopathology. Thalidomide / pharmacology. Thalidomide / therapeutic use. Vascular Endothelial Growth Factor A / antagonists & inhibitors. Vascular Endothelial Growth Factor A / blood
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Anti-Inflammatory Agents / therapeutic use. Dexamethasone / therapeutic use. Drug Administration Schedule. Drug Therapy, Combination. Female. Humans. Male. Middle Aged

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  • (PMID = 18469028.001).
  • [ISSN] 1468-330X
  • [Journal-full-title] Journal of neurology, neurosurgery, and psychiatry
  • [ISO-abbreviation] J. Neurol. Neurosurg. Psychiatry
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Anti-Inflammatory Agents; 0 / Vascular Endothelial Growth Factor A; 4Z8R6ORS6L / Thalidomide; 7S5I7G3JQL / Dexamethasone
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57. Iseri M, Ozturk M, Ulubil SA: Synchronous presentation of extramedullary plasmacytoma in the nasopharynx and the larynx. Ear Nose Throat J; 2009 Nov;88(11):E9-12
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  • She was treated with a combination of surgery, radiotherapy, and chemotherapy.
  • We discuss the clinical features and treatment of plasma cell neoplasms in general and their three variants in particular.
  • [MeSH-major] Laryngeal Neoplasms / diagnosis. Larynx / pathology. Nasopharyngeal Neoplasms / diagnosis. Nasopharynx / pathology. Plasmacytoma / diagnosis
  • [MeSH-minor] Endoscopy. Female. Humans. Laryngoscopy. Middle Aged. Prognosis. Time Factors

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  • (PMID = 19924656.001).
  • [ISSN] 1942-7522
  • [Journal-full-title] Ear, nose, & throat journal
  • [ISO-abbreviation] Ear Nose Throat J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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58. Lalive PH, Passweg JR, Kuntzer T: [Neuropathy associated with monoclonal gammopathy (dysglobulinemia)]. Rev Med Suisse; 2009 Apr 29;5(201):962-4, 966-7
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  • The clinical course is often chronic and progressive and requires a precise diagnosis of the type of plasma cell disorder associated with the neuropathy, to investigate other organs manifestations and to assess the presence of specific markers.
  • These steps are required to initiate an appropriate therapy that may include chemotherapy, immunosuppressive or immunomodulatory treatment.

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  • (PMID = 19476059.001).
  • [ISSN] 1660-9379
  • [Journal-full-title] Revue médicale suisse
  • [ISO-abbreviation] Rev Med Suisse
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Switzerland
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59. Renner S, Weisz J, Krajewski S, Krajewska M, Reed JC, Lichtenstein A: Expression of BAX in plasma cell dyscrasias. Clin Cancer Res; 2000 Jun;6(6):2371-80
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  • [Title] Expression of BAX in plasma cell dyscrasias.
  • Several studies demonstrate that the BCL-2 and BCL-XL antiapoptotic genes are variably expressed in plasma cells of patients with multiple myeloma (MM).
  • However, the plasma cell expression of BAX protein, their major proapoptotic partner, has not been investigated.
  • Our initial Western blot analysis of myeloma cell extracts also suggested patient variability in the expression of BAX, which was not altered by exposure to interleukin 6.
  • For correlations with outcome, expression was assessed in 43 patients at diagnosis who were treated with melphalan and prednisone; 30 at diagnosis who were treated with vincristine, Adriamycin, and dexamethasone; and 29 at relapse who were treated with second-line therapy.
  • There was no correlation between BAX or BCL-2 expression and response to chemotherapy or duration of response or between BCL-2 expression and survival.
  • However, patients who demonstrated extremely low plasma cell BAX expression had significantly increased survival.
  • BAX expression did not correlate with expression of proliferating cell nuclear antigen used as a marker of proliferation.
  • These data indicate a myeloma-specific increase in BAX expression in plasma cells and suggest that low BAX expression identifies a cohort of patients with long survival, which is not specifically associated with low proliferating cell nuclear antigen expression.
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Apoptosis. Biopsy. Blotting, Western. Bone Marrow / metabolism. Cell Division / drug effects. Dexamethasone / administration & dosage. Disease-Free Survival. Doxorubicin / administration & dosage. Humans. Immunohistochemistry. Melphalan / administration & dosage. Multiple Myeloma / metabolism. Multiple Myeloma / pathology. Plasma Cells / metabolism. Plasma Cells / pathology. Prednisone / administration & dosage. Proliferating Cell Nuclear Antigen / biosynthesis. Proto-Oncogene Proteins c-bcl-2 / biosynthesis. Recurrence. Time Factors. Vincristine / administration & dosage. bcl-2-Associated X Protein

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  • (PMID = 10873089.001).
  • [ISSN] 1078-0432
  • [Journal-full-title] Clinical cancer research : an official journal of the American Association for Cancer Research
  • [ISO-abbreviation] Clin. Cancer Res.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA 551164; United States / NCI NIH HHS / CA / CA 69381; United States / NCI NIH HHS / CA / U10CA2115
  • [Publication-type] Journal Article; Research Support, U.S. Gov't, Non-P.H.S.; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / BAX protein, human; 0 / Proliferating Cell Nuclear Antigen; 0 / Proto-Oncogene Proteins; 0 / Proto-Oncogene Proteins c-bcl-2; 0 / bcl-2-Associated X Protein; 5J49Q6B70F / Vincristine; 7S5I7G3JQL / Dexamethasone; 80168379AG / Doxorubicin; Q41OR9510P / Melphalan; VB0R961HZT / Prednisone
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60. Anguiano A, Tuchman SA, Acharya C, Salter K, Gasparetto C, Zhan F, Dhodapkar M, Nevins J, Barlogie B, Shaughnessy JD Jr, Potti A: Gene expression profiles of tumor biology provide a novel approach to prognosis and may guide the selection of therapeutic targets in multiple myeloma. J Clin Oncol; 2009 Sep 1;27(25):4197-203
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  • [Title] Gene expression profiles of tumor biology provide a novel approach to prognosis and may guide the selection of therapeutic targets in multiple myeloma.
  • PURPOSE: Monoclonal gammopathy of undetermined significance (MGUS) and multiple myeloma (MM) comprise heterogeneous disorders with incompletely understood molecular defects and variable clinical features.
  • METHODS: Using gene expression and clinical data from 877 patients ranging from normal plasma cells (NPC) to relapsed MM (RMM), we applied gene expression signatures reflecting deregulation of oncogenic pathways and tumor microenvironment to highlight molecular changes that occur as NPCs transition to MM, create a high-risk MGUS gene signature, and subgroup International Staging System (ISS) stages into more prognostically accurate clusters of patients.
  • Regarding MM, we subclassified ISS stages into clusters based on shared features of tumor biology.
  • These clusters differentiated themselves based on predictions for prognosis and chemotherapy sensitivity (eg, in ISS stage I, one cluster was characterized by increased CIN, cyclophosphamide resistance, and a poor prognosis).
  • CONCLUSION: GEP provides insight into the molecular defects underlying plasma cell dyscrasias that may explain their clinical heterogeneity.
  • GEP also may also refine current prognostic and therapeutic models for MGUS and MM.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Biomarkers, Tumor / genetics. Gene Expression Profiling / methods. Gene Expression Regulation, Neoplastic. Multiple Myeloma / genetics. Oligonucleotide Array Sequence Analysis. Paraproteinemias / genetics. Patient Selection
  • [MeSH-minor] Adult. Aged. Chromosomal Instability. Cluster Analysis. Databases as Topic. Disease-Free Survival. Female. Genotype. Humans. Kaplan-Meier Estimate. Male. Middle Aged. Neoplasm Staging. Phenotype. Predictive Value of Tests. Prognosis. Proto-Oncogene Proteins c-myc / genetics. Risk Assessment. Time Factors

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  • [ErratumIn] J Clin Oncol. 2012 Apr 20;30(12):1398
  • (PMID = 19636021.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / P01 CA055819
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Biomarkers, Tumor; 0 / MYC protein, human; 0 / Proto-Oncogene Proteins c-myc
  • [Other-IDs] NLM/ PMC4881369
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61. Sakakima M, Fujigaki Y, Tsuji T, Fukasawa H, Miyaji T, Naito K, Yamamoto T, Yonemura K, Ohnishi K, Hishida A: High dose chemotherapy and stem cell support in a patient of light- and heavy-chain deposition disease with abnormal marrow cell surface antigens and no monoclonal protein. Intern Med; 2005 Sep;44(9):970-4
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  • [Title] High dose chemotherapy and stem cell support in a patient of light- and heavy-chain deposition disease with abnormal marrow cell surface antigens and no monoclonal protein.
  • A 53-year-old man with nephrotic syndrome and severe renal failure was diagnosed with light- and heavy-chain deposition disease (LHCDD) by renal biopsy.
  • The patient had no monoclonal protein and mild marrow plasmacytosis (6%), but marrow plasma cells expressed CD19(-)CD56+ and predominant monoclonal kappa-chain, indicating plasma cell dyscrasia.
  • Conventional chemotherapy was ineffective and did not improve renal failure.
  • High dose chemotherapy/peripheral blood stem cell transplantation (HDC/PBSCT) was introduced even after hemodialysis to eliminate aberrant clone and normalization of bone marrow cell surface markers.
  • Immunophenotypic analysis of marrow cells facilitates clinical decision making regarding the use of HDC/PBSCT for LHCDD patients without monoclonal protein.
  • [MeSH-major] Heavy Chain Disease / therapy. Immunoglobulin Light Chains. Paraproteinemias / therapy
  • [MeSH-minor] Antigens, CD19 / metabolism. Antigens, CD56 / metabolism. Antineoplastic Agents / therapeutic use. Bone Marrow Cells / immunology. Combined Modality Therapy. Humans. Male. Middle Aged. Nephrotic Syndrome / drug therapy. Nephrotic Syndrome / therapy. Peripheral Blood Stem Cell Transplantation

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  • [CommentIn] Intern Med. 2005 Sep;44(9):915-6 [16258202.001]
  • (PMID = 16258214.001).
  • [ISSN] 0918-2918
  • [Journal-full-title] Internal medicine (Tokyo, Japan)
  • [ISO-abbreviation] Intern. Med.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antigens, CD19; 0 / Antigens, CD56; 0 / Antineoplastic Agents; 0 / Immunoglobulin Light Chains
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62. Turk HM, Komurcu S, Ozet A, Kuzhan O, Günhan O: An unusual presentation of extramedullary plasmacytoma in testis and review of the literature. Med Oncol; 2010 Dec;27(4):1378-80
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  • Extramedullary plasmacytoma is a rare plasma cell neoplasm, and it is extremely uncommon in the testicles.
  • He received chemotherapy, melphalan and prednisolone, and palliative radiotherapy.
  • He succumbed to disease after 8 months.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Multiple Myeloma / diagnosis. Orchiectomy. Plasmacytoma / diagnosis. Testicular Neoplasms / diagnosis

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  • (PMID = 20035386.001).
  • [ISSN] 1559-131X
  • [Journal-full-title] Medical oncology (Northwood, London, England)
  • [ISO-abbreviation] Med. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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63. Singhal S, Mehta J: Thalidomide in cancer. Biomed Pharmacother; 2002 Feb;56(1):4-12
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  • After its success in myeloma, it has been investigated in other plasma cell dyscrasias, myelodysplastic syndromes, gliomas, Kaposi's sarcoma, renal cell carcinoma, advanced breast cancer, and colon cancer.
  • Thalidomide causes responses in 30-50% of myeloma patients as a single agent, and acts synergistically with corticosteroids and chemotherapy.
  • Responses have also been seen in one-third of patients with Kaposi's sarcoma, in a small proportion of patients with renal cell carcinoma and high-grade glioma, and in some patients with colon cancer in combination with irinotecan.
  • The drug is being investigated currently in a number of clinical trials for cancer.
  • Thrombotic phenomena are especially common when thalidomide is combined with chemotherapy.
  • Adverse effects severe enough to necessitate cessation of therapy are seen in around 20% of patients.
  • A therapeutic trial of thalidomide is essential in all patients with relapsed or refractory myeloma.
  • In other cancers, the best way to use the drug is in the setting of clinical trials.
  • In the absence of access to studies or alternative therapeutic options, thalidomide could be considered singly or in combination with standard therapy.
  • [MeSH-major] Angiogenesis Inhibitors / therapeutic use. Neoplasms / drug therapy. Thalidomide / therapeutic use
  • [MeSH-minor] Bradycardia / chemically induced. Exanthema / chemically induced. Humans. Interleukin-2 / antagonists & inhibitors. Interleukin-6 / antagonists & inhibitors. Peripheral Nervous System Diseases / chemically induced. Thrombosis / chemically induced. Tumor Necrosis Factor-alpha / antagonists & inhibitors

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  • (PMID = 11905508.001).
  • [ISSN] 0753-3322
  • [Journal-full-title] Biomedicine & pharmacotherapy = Biomédecine & pharmacothérapie
  • [ISO-abbreviation] Biomed. Pharmacother.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Interleukin-2; 0 / Interleukin-6; 0 / Tumor Necrosis Factor-alpha; 4Z8R6ORS6L / Thalidomide
  • [Number-of-references] 61
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64. Oehadian A, Prasetya D, Fadjari TH: POEMS syndrome: a rare case of monoclonal plasmaproliferative disorder. Acta Med Indones; 2010 Apr;42(2):100-3
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  • [Title] POEMS syndrome: a rare case of monoclonal plasmaproliferative disorder.
  • POEMS syndrome is defined by the presence of a peripheral neuropathy (P), a monoclonal plasma cell disorder (M), and other paraneoplastic features, the most common of which include organomegaly (O), endocrinopathy (E), skin changes (S).
  • Not all features of the disease are required to make the diagnosis.
  • We report a case of POEMS syndrome in a 50-year-old female who presented with weakness, abdominal swelling and history of red cell transfusions.
  • Her peripheral blood smear did not show granulopoiesis maturation from myeloblast nor leukoerytroblastic feature which was characteristic of CML and myelofibrosis.
  • Bone marrow examination showed normal plasma cells.
  • Until then she continued on melphalan and prednisone treatment.
  • Although POEMS syndrome is a rare disease, it should be considered in patient with hepatosplenomegaly, especially if accompanied by peripheral neuropathy.
  • [MeSH-minor] Diagnosis, Differential. Drug Therapy, Combination. Electrophoresis. Female. Glucocorticoids / therapeutic use. Humans. Melphalan / therapeutic use. Middle Aged. Myeloablative Agonists / therapeutic use. Prednisone / therapeutic use

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  • (PMID = 20513935.001).
  • [ISSN] 0125-9326
  • [Journal-full-title] Acta medica Indonesiana
  • [ISO-abbreviation] Acta Med Indones
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Indonesia
  • [Chemical-registry-number] 0 / Glucocorticoids; 0 / Myeloablative Agonists; 0 / beta 2-Microglobulin; Q41OR9510P / Melphalan; VB0R961HZT / Prednisone
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65. Arkenau HT, Widjaja A: [A rare case of cholestasis and macrohematuria in a 52-year-old patient]. Med Klin (Munich); 2002 Aug 15;97(8):480-3
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  • INVESTIGATIONS AND THERAPY: Due to the ultrasound and the CT scan of the abdomen, a kidney tumor was suspected.
  • A plasma cell dyscrasia of 15% was found by bone marrow biopsy.
  • Immunfixation showed an IgG-kappa light chain in plasma and urine.
  • Thus, the patient had a plasmacytoma Stage II associated with IgG-kappa light chain AL-amyloidosis which was treated by chemotherapy (melphalan and prednisolone).
  • CONCLUSION: This unusual case presents a cholestatic liver disease due to bile duct obstruction secondary to amyloid deposits.
  • Although AL-amyloidosis is easily diagnosed by certain criteria, the disease is often recognized too late with consequently poor prognosis.

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  • (PMID = 12229247.001).
  • [ISSN] 0723-5003
  • [Journal-full-title] Medizinische Klinik (Munich, Germany : 1983)
  • [ISO-abbreviation] Med. Klin. (Munich)
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Amyloid; 0 / Immunoglobulin G; 0 / Immunoglobulin Light Chains; 0 / Immunoglobulin kappa-Chains; 0 / amyloid protein AL
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66. Jánosi J, Sebestyén A, Mikala G, Petö M, Jákó J, Domján G, Németh J, Kis Z, Kopper L, Vályi-Nagy I: [Soluble syndecan-1 levels in different plasma cell dyscrasias]. Orv Hetil; 2005 Jan 23;146(4):165-8
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  • [Title] [Soluble syndecan-1 levels in different plasma cell dyscrasias].
  • [Transliterated title] Szolúbilis syndecan-1-(CD 138)-koncentráció plazmasejt-dyscrasiákban.
  • INTRODUCTION: Syndecans are a family of cell surface proteoglycans.
  • In the bone marrow of multiple myeloma patients syndecan-1 is expressed only on the surface of malignant plasma cells.
  • The aim of the study was to determine the soluble syndecan-1 levels in different plasma cell dyscrasias.
  • In addition to these findings a statistical correlation with other independent prognostic factors such as serum beta2-microglobulin level, monoclonal immunoglobulin concentration, and bone marrow plasma cell count could also be noted.
  • A significant decrease in median serum syndecan level was observed in patients who responded to chemotherapy, whereas no change in the median syndecan-1 level could be observed in nonresponders.
  • CONCLUSION: These findings confirm the observation that high serum soluble syndecan-1 level is associated with a more advanced disease stage and is a strong independent indicator of poor prognosis.
  • A diminished serum syndecan-1 reading as a result of chemotherapy may be a good indicator of favorable response to antitumor treatment.

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  • (PMID = 15751511.001).
  • [ISSN] 0030-6002
  • [Journal-full-title] Orvosi hetilap
  • [ISO-abbreviation] Orv Hetil
  • [Language] hun
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Hungary
  • [Chemical-registry-number] 0 / Membrane Glycoproteins; 0 / Proteoglycans; 0 / SDC1 protein, human; 0 / Syndecan-1; 0 / Syndecans
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67. Etienne G, Grenouillet M, Ghiringhelli C, Vatan R, Lazaro E, Germain P, Mercié P, Longy-Boursier M: [Pulmonary plasmacytoma: about two new cases and review of the literature]. Rev Med Interne; 2004 Aug;25(8):591-5
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  • [Transliterated title] Plasmocytome pulmonaire: à propos de deux nouvelles observations et revue de la littérature.
  • INTRODUCTION: Extramedullary plasmacytoma is an uncommon plasma cell malignancy mainly located to the upper aerodigestive tract.
  • Five patients (14%) have developed multiple myeloma within 3 years following plasmacytoma diagnostic.
  • In spite of sustained responses with radiotherapy or chemotherapy, surgical resection while feasible remains the first therapeutic option.

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  • (PMID = 15276291.001).
  • [ISSN] 0248-8663
  • [Journal-full-title] La Revue de medecine interne
  • [ISO-abbreviation] Rev Med Interne
  • [Language] FRE
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Number-of-references] 42
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68. Basak GW, Srivastava AS, Malhotra R, Carrier E: Multiple myeloma bone marrow niche. Curr Pharm Biotechnol; 2009 Apr;10(3):345-6
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  • MM is a neoplastic plasma cell disorder which not only resides in BM but also converts it into specialized neoplastic niche.
  • This niche aids the growth and spreading of tumor cells by a complex interplay of cytokines, chemokines, proteolytic enzymes and adhesion molecules.
  • Moreover, the MM BM microenvironment was shown to confer survival and chemoresistance of MM cells to current therapies.
  • Therefore, there is a strong need to further dissect the MM BM niche and understand the process of how the complex interactions with BM milieu influence MM growth, survival and development of resistance to chemotherapy.
  • A better and more detailed understanding of neoplastic MM niche will provide a guiding model for identifying and validating novel targeted therapies directed against MM.

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  • (PMID = 19355944.001).
  • [ISSN] 1873-4316
  • [Journal-full-title] Current pharmaceutical biotechnology
  • [ISO-abbreviation] Curr Pharm Biotechnol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Netherlands
  • [Number-of-references] 101
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69. Lin CK, Sung YC: Newly diagnosed multiple myeloma in Taiwan: the evolution of therapy, stem cell transplantation and new treatment agents. Hematol Oncol Stem Cell Ther; 2009;2(3):385-93
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  • [Title] Newly diagnosed multiple myeloma in Taiwan: the evolution of therapy, stem cell transplantation and new treatment agents.
  • Multiple myeloma is a clonal plasma cell dyscrasia with clinical heterogeneity.
  • One is whether the patient is a candidate for high-dose chemotherapy with stem cell support and the other is risk stratification.
  • As novel therapeutics have emerged, it is increasingly important to introduce a risk-adapted approach.
  • The heterogeneity of the disease is established, for the most part, by disease biology, predominantly genetics.
  • This group of patients may not respond well to high-dose chemotherapy and require early introduction of newer treatments such as the bortezomib-containing regimen.
  • The main factor in determining the eligibility for stem cell transplants is age.
  • Based on the current literature and situation in Taiwan, we suggest stem cell transplantation if the patient is younger than 55 years of age.
  • Finally, we have also reviewed the status and the treatment of multiple myeloma in Taiwan.
  • Fortunately, there has been an improvement in awareness, diagnosis and treatment.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Multiple Myeloma / therapy. Stem Cell Transplantation
  • [MeSH-minor] Combined Modality Therapy. Humans. Taiwan


70. Huang W, Cao D, Ma J, Yang X, Xiao J, Zheng W, Feng D, Wu Z, Huang Q, Chen D, Jia L: Solitary plasmacytoma of cervical spine: treatment and prognosis in patients with neurological lesions and spinal instability. Spine (Phila Pa 1976); 2010 Apr 15;35(8):E278-84
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  • [Title] Solitary plasmacytoma of cervical spine: treatment and prognosis in patients with neurological lesions and spinal instability.
  • STUDY DESIGN: A consecutive series of 19 cases of solitary plasmacytoma (SP) of cervical spine that underwent surgical treatment and one case that underwent pure radiotherapy were observed from 1995 to 2006.
  • OBJECTIVE: To discuss the clinical characteristics, therapeutic methods and factors affecting prognosis of SP in the cervical spine.
  • SUMMARY OF BACKGROUND DATA: SP of bone is a rare plasma cell tumor which represents the proliferation of monoclonal plasma cells without evidence of significant bone marrow plasma cell infiltration.
  • Although radical radiotherapy is the treatment of choice for SP of the bone, recommendations for treatment methods of this disease have been solely based on limited data from retrospective studies.
  • There were 13 men and 7 women ranging in age from 32 to 76 years with a mean of 56 years.
  • All surgery patients received radiotherapy as adjunctive therapy postoperatively.
  • RESULTS: Follow-up of the 20 patients ranged from 25 to 132 months with a mean of 61 months.
  • The other 15 patients achieved disease-free survival after surgery with adjunctive radiotherapy.
  • No significant abnormality was detected on M protein, bone marrow aspiration, and emission computed tomography or positron emission tomography/computed tomography examinations.
  • Gross total tumor resection or total spondylectomy by piecemeal manner with adjuvant radiotherapy can markedly reduce local recurrences and lower the possibility of progressing to MM.
  • Patients with progression to MM should be treated with individualized chemotherapy, but the prognosis may be poor.
  • [MeSH-major] Cervical Vertebrae / surgery. Neurosurgical Procedures / methods. Plasmacytoma / radiotherapy. Plasmacytoma / surgery. Spinal Neoplasms / radiotherapy. Spinal Neoplasms / surgery
  • [MeSH-minor] Adult. Aged. Bone Transplantation / methods. Combined Modality Therapy / methods. Combined Modality Therapy / statistics & numerical data. Female. Humans. Internal Fixators. Male. Middle Aged. Postoperative Complications / epidemiology. Prosthesis Implantation / methods. Radiotherapy / methods. Radiotherapy / statistics & numerical data. Reconstructive Surgical Procedures. Retrospective Studies. Survival Rate. Treatment Outcome

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  • (PMID = 20228695.001).
  • [ISSN] 1528-1159
  • [Journal-full-title] Spine
  • [ISO-abbreviation] Spine
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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71. Dispenzieri A, Gertz MA: Treatment options for POEMS syndrome. Expert Opin Pharmacother; 2005 Jun;6(6):945-53
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Treatment options for POEMS syndrome.
  • POEMS syndrome is a rare paraneoplastic syndrome secondary to a plasma cell dyscrasia.
  • Recognition of the complex of a combination of peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasmaproliferative disorder, skin changes, sclerotic bone lesions, Castleman's disease, thrombocytosis, papilledema, peripheral oedema, pleural effusions, ascites, fingernail clubbing and white nails, is the first step in effectively managing the disease.
  • In patients with a dominant sclerotic plasmacytoma, first-line therapy should include radiation to the lesion.
  • Retrospective analysis and personal experience would dictate that systemic therapy be considered for patients with diffuse sclerotic lesions or absence of any bone lesion, and for those who have not demonstrated stabilisation of their disease 3-6 months after completing radiation therapy.
  • For those patients with diffuse disease, systemic therapy is indicated.
  • Useful approaches include therapy with corticosteroids, low dose alkylator therapy and high dose chemotherapy with peripheral blood stem cell transplant.
  • Until the pathogenesis is fully understood, these are the mainstays of treatment for patients with POEMS syndrome.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. POEMS Syndrome / drug therapy. POEMS Syndrome / surgery
  • [MeSH-minor] Humans. Plasmapheresis / trends. Stem Cell Transplantation / trends. Survival Rate

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  • (PMID = 15952922.001).
  • [ISSN] 1744-7666
  • [Journal-full-title] Expert opinion on pharmacotherapy
  • [ISO-abbreviation] Expert Opin Pharmacother
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 56
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72. Ardashev VN, Potekhin NP, Rukavitsyn OA, Borisov AG, Malysheva SA: [A case of primary amyloidosis]. Klin Med (Mosk); 2006;84(6):56-9
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  • Diagnostics of primary (AL) amyloidosis is difficult enough; treatment of this disease in not less difficult or more adequate.
  • Because of similarity of the pathogenesis of AL-amyloidosis and that of multiple myeloma, similar therapeutic regimens, directed towards depression of plasma cell dyscrasia, are used in both cases: administration of melphalan in various doses together with prednisolone, administration of vincristine, adriablastine and dexamethasone, as well as high-dose chemotherapy with melphalan and autologic stem cell transplantation.
  • This therapeutic approach makes it possible to reach clinico-laboratory remission and prolong the life of patients with AL-amyloidosis.
  • The article contains a case description of a patient with AL-amyloidosis, who underwent a successful high-dose melphalan therapy with subsequent autologic stem cell transplantation.
  • [MeSH-major] Amyloidosis / diagnosis. Amyloidosis / pathology. Antineoplastic Agents, Alkylating / therapeutic use. Melphalan / therapeutic use
  • [MeSH-minor] Diagnosis, Differential. Drug Therapy, Combination. Humans. Immunoglobulin G / immunology. Male. Middle Aged. Prednisolone / therapeutic use

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  • (PMID = 16875072.001).
  • [ISSN] 0023-2149
  • [Journal-full-title] Klinicheskaia meditsina
  • [ISO-abbreviation] Klin Med (Mosk)
  • [Language] rus
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Russia (Federation)
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 0 / Immunoglobulin G; 9PHQ9Y1OLM / Prednisolone; Q41OR9510P / Melphalan
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73. Tassone P, Tagliaferri P, Fulciniti MT, Di Martino MT, Venuta S: Novel therapeutic approaches based on the targeting of microenvironment-derived survival pathways in human cancer: experimental models and translational issues. Curr Pharm Des; 2007;13(5):487-96
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  • [Title] Novel therapeutic approaches based on the targeting of microenvironment-derived survival pathways in human cancer: experimental models and translational issues.
  • It is a current idea that carcinogenesis as well as tumor progression are dynamic processes, which involve inherited as well as somatic mutations and include a continuing adaptation to different microenvironmental conditions.
  • There is, in fact, rising evidence that tumor cells are under a persistent stress and that autocrine as well as microenvironment-derived survival factors play a substantial role for the final outcome of the tumor development as well as for response to the anti-tumor therapy.
  • By the use of plasma cell disorders as an outstanding clinical model, we will discuss the development of novel in vivo preclinical models which recapitulate the human bone marrow milieu.
  • [MeSH-major] Cell Communication / drug effects. Cell Transformation, Neoplastic / drug effects. Cell Transformation, Neoplastic / pathology. Disease Models, Animal. Drug Delivery Systems / methods. Protein Biosynthesis / drug effects. Signal Transduction / drug effects. Xenograft Model Antitumor Assays / methods
  • [MeSH-minor] Animals. Cell Survival / drug effects. Cell Survival / genetics. Humans

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  • (PMID = 17348845.001).
  • [ISSN] 1873-4286
  • [Journal-full-title] Current pharmaceutical design
  • [ISO-abbreviation] Curr. Pharm. Des.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Netherlands
  • [Number-of-references] 106
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74. Dimopoulos MA, Kastritis E, Rajkumar SV: Treatment of plasma cell dyscrasias with lenalidomide. Leukemia; 2008 Jul;22(7):1343-53
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  • [Title] Treatment of plasma cell dyscrasias with lenalidomide.
  • Lenalidomide is an immunomodulatory drug, structurally related to thalidomide, with pleiotropic activity including antiangiogenic and antineoplastic properties.
  • Subsequent phase II and III studies confirmed the activity of lenalidomide either as a single agent or in combination with dexamethasone in relapsed or refractory myeloma patients, whereas combinations with chemotherapy induce high response rates and durable remissions.
  • Lenalidomide has been used successfully as an upfront treatment either with high or low dose dexamethasone or with melphalan and prednisone, resulting in high overall response and complete response rates and excellent 1-year survival.
  • In this review, we summarize the mechanisms of action, toxicity and clinical activity, and the current role of lenalidomide in patients with multiple myeloma or other related plasma cell disorders.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Paraproteinemias / drug therapy. Thalidomide / analogs & derivatives
  • [MeSH-minor] Amyloidosis / drug therapy. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Marrow / drug effects. Boronic Acids / administration & dosage. Bortezomib. Clinical Trials as Topic. Dexamethasone / administration & dosage. Humans. Multiple Myeloma / drug therapy. Pyrazines / administration & dosage. Renal Insufficiency / metabolism. Thromboembolism / chemically induced. Waldenstrom Macroglobulinemia / drug therapy

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  • (PMID = 18509355.001).
  • [ISSN] 1476-5551
  • [Journal-full-title] Leukemia
  • [ISO-abbreviation] Leukemia
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA10080; United States / NCI NIH HHS / CA / CA107476; United States / NCI NIH HHS / CA / CA62242; United States / NCI NIH HHS / CA / CA93842
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Boronic Acids; 0 / Pyrazines; 4Z8R6ORS6L / Thalidomide; 69G8BD63PP / Bortezomib; 7S5I7G3JQL / Dexamethasone; F0P408N6V4 / lenalidomide
  • [Number-of-references] 86
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75. Hussein MA: Thromboembolism risk reduction in multiple myeloma patients treated with immunomodulatory drug combinations. Thromb Haemost; 2006 Jun;95(6):924-30
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  • [Title] Thromboembolism risk reduction in multiple myeloma patients treated with immunomodulatory drug combinations.
  • Certain solid tumors and hematologic malignancies impose an inherently elevated risk of VTE that is compounded by chemotherapy and other risk factors.
  • Multiple myeloma (MM) and other plasma cell dyscrasias are thrombogenic as a consequence of their multiple hemostatic effects, including elevated interleukin-6 levels, pro-coagulant antibody formation, paraprotein interference with fibrin structure, activated protein C resistance, and endothelial damage.
  • The oral immunomodulatory drugs thalidomide and lenalidomide have produced major therapeutic responses in patients with MM when used in combination with oral steroids and chemotherapy, but a high incidence of VTE has been reported.
  • This review discusses emerging results on the use of VTE prophylaxis to minimize VTE risks associated with MM treatment regimens containing thalidomide and lenalidomide.
  • [MeSH-major] Anticoagulants / therapeutic use. Embolism / prevention & control. Immunologic Factors / adverse effects. Multiple Myeloma / drug therapy
  • [MeSH-minor] Aspirin / therapeutic use. Clinical Trials as Topic. Drug Therapy, Combination. Factor V / genetics. Heparin, Low-Molecular-Weight / therapeutic use. Humans. Mutation. Prothrombin / genetics. Risk Reduction Behavior. Treatment Outcome. Venous Thrombosis / etiology. Venous Thrombosis / genetics. Venous Thrombosis / prevention & control. Warfarin / therapeutic use


76. Pentenero M, Davico Bonino L, Tomasini C, Conrotto D, Gandolfo S: Localized oral amyloidosis of the palate. Amyloid; 2006 Mar;13(1):42-6
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  • BACKGROUND: Amyloidosis is a rare disease with multifactorial pathogenesis.
  • CONCLUSIONS: The presence of systemic amyloidosis or underlying plasma cell dyscrasia have to be ruled out in patients presenting with a diagnosis of amyloidosis of the oral mucosa.
  • If a primary localized amyloidosis is proven, the surgical therapy may be useful to eliminate a functional impairment.
  • [MeSH-minor] Aged. Antifungal Agents / therapeutic use. Candidiasis / drug therapy. Candidiasis / pathology. Female. Humans. Mouth Mucosa / microbiology. Mouth Mucosa / pathology

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  • (PMID = 16690500.001).
  • [ISSN] 1350-6129
  • [Journal-full-title] Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis
  • [ISO-abbreviation] Amyloid
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antifungal Agents
  • [Number-of-references] 29
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77. Skinner M, Sanchorawala V, Seldin DC, Dember LM, Falk RH, Berk JL, Anderson JJ, O'Hara C, Finn KT, Libbey CA, Wiesman J, Quillen K, Swan N, Wright DG: High-dose melphalan and autologous stem-cell transplantation in patients with AL amyloidosis: an 8-year study. Ann Intern Med; 2004 Jan 20;140(2):85-93
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  • [Title] High-dose melphalan and autologous stem-cell transplantation in patients with AL amyloidosis: an 8-year study.
  • BACKGROUND: AL amyloidosis is a fatal disease resulting from tissue deposition of amyloid fibrils derived from monoclonal immunoglobulin light chains.
  • Treatment with oral chemotherapy is minimally effective.
  • OBJECTIVE: To test survival and organ response in a large sample of patients treated with high-dose intravenous melphalan (100 to 200 mg/m2) and autologous blood stem-cell transplantation.
  • INTERVENTION: High-dose chemotherapy and autologous stem-cell transplantation for patients who met eligibility requirements based on organ involvement and clinical status.
  • MEASUREMENTS: Survival analysis of all patients evaluated and a detailed analysis of treatment outcome in the subgroup that received high-dose melphalan and stem-cell transplantation.
  • RESULTS: Among 701 patients with AL amyloidosis, 394 (56%) were eligible for high-dose melphalan and stem-cell transplantation; 82 did not proceed with treatment because of patient choice or disease progression.
  • Median survival of the 312 patients who initiated treatment was 4.6 years.
  • A complete hematologic response, defined as no evidence of an underlying plasma cell dyscrasia 1 year after treatment, was achieved in 40% of patients and was associated with prolonged survival.
  • Statistically significant improvements occurred in end-organ disease and were greater in patients with a complete hematologic response.
  • Mortality rate within 100 days of treatment with high-dose melphalan and stem-cell transplantation was 13%; patients with cardiomyopathy had the highest mortality rates.
  • CONCLUSIONS: Treatment of selected patients with AL amyloidosis by using high-dose melphalan and stem-cell transplantation resulted in hematologic remission, improved 5-year survival, and reversal of amyloid-related disease in a substantial proportion.
  • [MeSH-major] Amyloidosis / therapy. Melphalan / administration & dosage. Stem Cell Transplantation


78. Gertz MA, Lacy MQ, Dispenzieri A, Hayman SR: Amyloidosis. Best Pract Res Clin Haematol; 2005;18(4):709-27
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  • Amyloidosis is an uncommon plasma-cell dyscrasia with an incidence of eight patients per million per year.
  • Newly developed therapeutic strategies, involving high-dose and intermediate-dose chemotherapy, have evolved in the last 3 years.
  • This paper reviews a diagnostic pathway clinicians can use to diagnose the disorder, assess a patient's prognosis, and logically plan a therapeutic strategy.
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Humans. Plasma Cells / pathology. Prognosis. Survival Analysis

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  • (PMID = 16026746.001).
  • [ISSN] 1521-6926
  • [Journal-full-title] Best practice & research. Clinical haematology
  • [ISO-abbreviation] Best Pract Res Clin Haematol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 79
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79. Orlowski RZ, Zeger EL: Targeting the proteasome as a therapeutic strategy against haematological malignancies. Expert Opin Investig Drugs; 2006 Feb;15(2):117-30
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  • [Title] Targeting the proteasome as a therapeutic strategy against haematological malignancies.
  • Inhibition of the proteasome induces beneficial antitumour effects by blocking cell-cycle progression, inducing apoptosis and suppressing angiogenesis.
  • Testing is ongoing to determine bortezomib's role in front-line therapy of this plasma cell dyscrasia, as well as in non-Hodgkin's lymphoma, in which encouraging single-agent activity has been seen.
  • [MeSH-major] Antineoplastic Agents / administration & dosage. Drug Delivery Systems / methods. Hematologic Neoplasms / drug therapy. Hematologic Neoplasms / enzymology. Protease Inhibitors / administration & dosage. Proteasome Inhibitors

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  • (PMID = 16433592.001).
  • [ISSN] 1744-7658
  • [Journal-full-title] Expert opinion on investigational drugs
  • [ISO-abbreviation] Expert Opin Investig Drugs
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / R01 CA102278; United States / NHLBI NIH HHS / HL / T32 HL 007149
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Boronic Acids; 0 / Protease Inhibitors; 0 / Proteasome Inhibitors; 0 / Pyrazines; 69G8BD63PP / Bortezomib; EC 3.4.25.1 / Proteasome Endopeptidase Complex
  • [Number-of-references] 71
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80. Raje N, Anderson KC: Multiple myeloma. Curr Treat Options Oncol; 2000 Apr;1(1):73-82
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  • Multiple myeloma (MM) is an incurable plasma cell dyscrasia that remains fatal.
  • Despite efforts over the past 3 to 4 decades, the median survival of patients with MM does not exceed 3 to 4 years.
  • Although patients receiving combination chemotherapy have higher response rates compared with those receiving oral melphalan and prednisolone, they have no survival advantage.
  • High-dose chemotherapy followed by autologous stem cell transplantation has documented benefit over conventional treatment and is currently the accepted mode of treatment for symptomatic MM.
  • Allogeneic transplantation is associated with high complete remission rates, but at the cost of high therapy-related mortality.
  • Maintenance treatment with interferon-alpha shows benefit, albeit in a small fraction of MM patients.
  • The use of bisphosphonates in patients with MM has clearly demonstrated benefit and reduced morbidity associated with bone disease.
  • All of these measures have improved remission rates and survival, but all patients with MM ultimately relapse and succumb to their disease.
  • Novel therapeutic strategies are therefore required to improve outcome of MM patients.
  • Immune-based strategies, including both adoptive immunotherapy and vaccinations, are currently being investigated in the preclinical and clinical setting, with the goal of enhancing autologous and allogeneic anti-MM immunity for therapeutic applications.
  • [MeSH-major] Multiple Myeloma / therapy
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Bone Marrow Transplantation. Clinical Trials as Topic. Combined Modality Therapy. Humans. Interferons / therapeutic use. Prognosis. Thalidomide / therapeutic use. Transplantation, Autologous

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  • (PMID = 12057063.001).
  • [ISSN] 1527-2729
  • [Journal-full-title] Current treatment options in oncology
  • [ISO-abbreviation] Curr Treat Options Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 4Z8R6ORS6L / Thalidomide; 9008-11-1 / Interferons
  • [Number-of-references] 39
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81. Jurisic V, Colovic N, Konjevic G, Minic I, Colovic M: An aggressive extramedullary cutaneous plasmacytoma associated with extreme alterations in the innate immune system. Onkologie; 2010;33(3):113-5
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  • BACKGROUND: The role of natural killer (NK) cells in plasma cell diseases has not yet been fully characterized.
  • CASE REPORT: We present the case of a 47-year-old man with an extremely aggressive extramedullary plasmacytoma of the lung with associated cutaneous lesions, whose disease was accompanied by a significantly decreased number of NK cells (CD56+, CD16+, CD3-) in the peripheral blood, very low NK cell activity levels, and a decreased interleukin-2 production from cultured cells in vitro.
  • Histology and immunohistochemistry of the lung and cutaneous lesions identified that the tumor was composed of clonal plasma cells which were CD38+++, CD138+++, lambda chain+, kappa chain-, and cytokeratin-.
  • The disease progressed rapidly despite local radiotherapy and systemic chemotherapy, and the patient died shortly after diagnosis.
  • CONCLUSIONS: Cutaneous involvement in extramedullary plasmacytoma represents a clinically aggressive variant of plasma cell tumor, which runs a rapid course and has associated devastating effects on the patient's innate immune system.
  • [MeSH-major] Immune System Diseases / complications. Immune System Diseases / pathology. Killer Cells, Natural / pathology. Plasmacytoma / complications. Plasmacytoma / pathology. Skin Neoplasms / complications. Skin Neoplasms / pathology

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  • [Copyright] Copyright 2010 S. Karger AG, Basel.
  • (PMID = 20215803.001).
  • [ISSN] 1423-0240
  • [Journal-full-title] Onkologie
  • [ISO-abbreviation] Onkologie
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Switzerland
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82. Wiesenthal AA, Nguyen BD: F-18 FDG PET/CT staging of multiple myeloma with diffuse osseous and extramedullary lesions. Clin Nucl Med; 2007 Oct;32(10):797-801
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  • Multiple myeloma is the most common plasma cell neoplasm, with abnormal clonal proliferation of B cells in bone marrow.
  • Its staging is important for therapeutic management and prognosis.
  • Through imaging integration, PET provides functional detection of high metabolic lesions whereas CT provides correlated anatomic localization.
  • The authors present a case of multiple myeloma status post chemotherapy and stem cell transplant with diffuse osseous and extramedullary lesions evaluated by PET/CT.
  • [MeSH-major] Bone Neoplasms / diagnosis. Fluorodeoxyglucose F18. Multiple Myeloma / diagnosis. Positron-Emission Tomography / methods. Soft Tissue Neoplasms / diagnosis. Tomography, X-Ray Computed / methods
  • [MeSH-minor] Humans. Male. Middle Aged. Neoplasm Staging. Radiopharmaceuticals. Subtraction Technique

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  • (PMID = 17885362.001).
  • [ISSN] 0363-9762
  • [Journal-full-title] Clinical nuclear medicine
  • [ISO-abbreviation] Clin Nucl Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
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83. Pratibha CB, Sreenivas V, Babu MK, Rout P, Nayar RC: Plasmacytoma of larynx--a case report. J Voice; 2009 Nov;23(6):735-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Plasma cell myeloma, the most common plasma cell neoplasm, is characterized by the presence of multiple lesions in the bone marrow.
  • A single isolated lesion may occur either in bone (solitary plasmacytoma of bone) or in soft tissue (extramedullary plasmacytoma).
  • A detailed evaluation for lesions at other sites is recommended as extramedullary plasmacytoma treated by radiation therapy has better survival rates than plasma cell myeloma, which is treated by chemotherapy.
  • A case of plasmacytoma of the larynx is presented highlighting clinical and histological features with a review of literature.
  • [MeSH-major] Laryngeal Neoplasms / pathology. Plasmacytoma / pathology
  • [MeSH-minor] Diagnosis, Differential. Follow-Up Studies. Humans. Immunohistochemistry. Laryngoscopy. Larynx / pathology. Male. Middle Aged. Treatment Outcome. Vocal Cords / pathology

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  • (PMID = 18619786.001).
  • [ISSN] 1873-4588
  • [Journal-full-title] Journal of voice : official journal of the Voice Foundation
  • [ISO-abbreviation] J Voice
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 19
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84. Singh D, Wadhwa J, Kumar L, Raina V, Agarwal A, Kochupillai V: POEMS syndrome: experience with fourteen cases. Leuk Lymphoma; 2003 Oct;44(10):1749-52
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  • The POEMS syndrome, also known as Crow-Fukase disease, is a rare multisystem disorder, which may take several years to evolve fully.
  • Herein, we report a series of 14 cases with POEMS syndrome at our centre over the past 8 years, which were analysed retrospectively for their clinical features, response to therapy and treatment outcome.
  • Presence of plasma cell dyscrasia (PCD) was essential for inclusion in this study.
  • Patients were excluded from study if there was a secondary cause of polyneuropathy like amyloidosis, drugs like vincristine, nerve root or spinal cord compression.
  • An association with Castleman's disease and vasculitis was also noted.
  • With different chemotherapy protocols, all treated patients (n = 12), had significant symptomatic improvement with or without objective improvement at median follow up of 48 months (range 6-120).
  • [MeSH-minor] Adult. Aged. Female. Humans. India. Male. Middle Aged. Paraproteinemias / diagnosis. Paraproteinemias / therapy. Retrospective Studies

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  • (PMID = 14692529.001).
  • [ISSN] 1042-8194
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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85. Kamezaki K, Nagafuji K: [POEMS syndrome: monoclonal plasma cell disorder with polyneuropathy]. Nihon Rinsho; 2007 Dec;65(12):2235-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [POEMS syndrome: monoclonal plasma cell disorder with polyneuropathy].
  • Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome is a rare multi-organ disease.
  • Patients without isolated plasmacytoma require systemic chemotherapy, which is ineffective in many cases.
  • In recent reports, high dose chemotherapy followed by autologous peripheral blood stem cell transplantation can dramatically improve clinical manifestations, particularly for polyneuropathy in such patients refractory for standard chemotherapy.
  • [MeSH-minor] Antibodies, Monoclonal / therapeutic use. Antibodies, Monoclonal, Humanized. Bevacizumab. Biomarkers / blood. Diagnosis, Differential. Drug Therapy, Combination. Hematopoietic Stem Cell Transplantation. Humans. Melphalan / administration & dosage. Prednisolone / administration & dosage. Thalidomide / therapeutic use. Vascular Endothelial Growth Factor A / blood

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  • (PMID = 18069266.001).
  • [ISSN] 0047-1852
  • [Journal-full-title] Nihon rinsho. Japanese journal of clinical medicine
  • [ISO-abbreviation] Nippon Rinsho
  • [Language] jpn
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / Biomarkers; 0 / Vascular Endothelial Growth Factor A; 2S9ZZM9Q9V / Bevacizumab; 4Z8R6ORS6L / Thalidomide; 9PHQ9Y1OLM / Prednisolone; Q41OR9510P / Melphalan
  • [Number-of-references] 12
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86. Seldin DC, Choufani EB, Dember LM, Wiesman JF, Berk JL, Falk RH, O'Hara C, Fennessey S, Finn KT, Wright DG, Skinner M, Sanchorawala V: Tolerability and efficacy of thalidomide for the treatment of patients with light chain-associated (AL) amyloidosis. Clin Lymphoma; 2003 Mar;3(4):241-6
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  • [Title] Tolerability and efficacy of thalidomide for the treatment of patients with light chain-associated (AL) amyloidosis.
  • Thalidomide is an effective therapy for multiple myeloma, although its mechanisms of action remain unclear.
  • Light chain-associated (AL) amyloidosis is a plasma cell disorder related to multiple myeloma, but in AL amyloidosis, fibrillar tissue deposits of clonal immunoglobulin light chains produce organ dysfunction.
  • To test the toxicity and efficacy of thalidomide in AL amyloidosis we initiated a phase I/II trial for patients with AL amyloidosis, most of whom had failed prior therapy with high-dose melphalan and autologous stem cell transplantation.
  • Sixteen patients were enrolled in the study with a median age of 62 years (range, 37-70 years).
  • Fourteen patients had renal involvement, 4 had cardiac involvement, 4 had liver involvement, and 2 had predominant soft tissue or lymph node involvement.
  • Side effects not frequently reported for other patient populations included exacerbation of peripheral and pulmonary edema and worsening azotemia.
  • In all, 50% of patients experienced grade 3/4 toxicity, and 25% had to discontinue the study drug.
  • [MeSH-major] Amyloidosis / drug therapy. Immunoglobulin Light Chains / immunology. Immunosuppressive Agents / administration & dosage. Paraproteinemias / drug therapy. Thalidomide / administration & dosage
  • [MeSH-minor] Adult. Aged. Female. Humans. Male. Maximum Tolerated Dose. Middle Aged. Prognosis. Proteinuria / etiology. Salvage Therapy. Treatment Outcome

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  • [CommentIn] Clin Lymphoma. 2003 Mar;3(4):247-8 [12672275.001]
  • (PMID = 12672274.001).
  • [ISSN] 1526-9655
  • [Journal-full-title] Clinical lymphoma
  • [ISO-abbreviation] Clin Lymphoma
  • [Language] eng
  • [Publication-type] Clinical Trial; Clinical Trial, Phase I; Clinical Trial, Phase II; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunoglobulin Light Chains; 0 / Immunosuppressive Agents; 4Z8R6ORS6L / Thalidomide
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87. Wilson WH, Hernandez-Ilizaliturri FJ, Dunleavy K, Little RF, O'Connor OA: Novel disease targets and management approaches for diffuse large B-cell lymphoma. Leuk Lymphoma; 2010 Aug;51 Suppl 1:1-10
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  • [Title] Novel disease targets and management approaches for diffuse large B-cell lymphoma.
  • Diffuse large B-cell lymphoma (DLBCL) responds well to treatment with CHOP and the R-CHOP regimen, but a subset of patients still fail to achieve complete or durable responses.
  • Recent advances in gene expression profiling have led to the identification of three different subtypes of DLBCL, and confirmed that patients with the activated B-cell (ABC) disease subtype are less likely to respond well to CHOP-based regimens than those with germinal centre B-cell-type (GCB) disease.
  • This discovery could herald the use of gene expression profiling to aid treatment decisions in DLBCL, and help identify the most effective management strategies for patients.
  • Treatment options for patients with relapsed or refractory DLBCL are limited and several novel agents are being developed to address this unmet clinical need.
  • Novel agents developed to treat plasma cell disorders such as multiple myeloma have shown promising activity in patients with NHL.
  • Indeed, the immunomodulatory agent lenalidomide and the proteasome inhibitors bortezomib and carfilzomib, as single agents or in combination with chemotherapy, have already demonstrated promising activity in patients with the ABC subtype of DLBCL.
  • One should not be complacent however when applying these agents to new disease types, because dose and drug scheduling can have marked effects on the responses achieved with investigational agents.
  • As more targeted agents are developed, the timing of administration with other agents in clinical trials will become increasingly important to ensure maximal efficacy while minimizing side effects.
  • [MeSH-major] Lymphoma, Large B-Cell, Diffuse / diagnosis. Lymphoma, Large B-Cell, Diffuse / drug therapy
  • [MeSH-minor] Disease Management. Gene Expression Profiling / classification. Humans. Precision Medicine / methods


88. Gil L, Komarnicki M: [Autologous stem cell transplantation in the treatment of primary systemic amyloidosis]. Pol Merkur Lekarski; 2009 Sep;27(159):181-4
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  • [Title] [Autologous stem cell transplantation in the treatment of primary systemic amyloidosis].
  • Primary systemic amyloidosis is a plasma cell dyscrasia resulting multiorgan failure and death.
  • The optimal treatment method is unknown.
  • Current status of therapy for primary amyloidosis based on has been publication analysis.
  • Treatment options for primary amyloidosis are similar to multiple myleoma therapy.
  • Treatment of primary amyloidosis should be based on risk factors analysis.
  • In selected patients high-dose therapy and autologous stem cell transplantation has been associated with higher response rate than standard chemotherapy However, autologous transplantation for primary amyloidosis remains controversial because of high treatment-related mortality.
  • Novel non-transplant methods for primary amyloidosis therapy are highly effective.
  • Early and detailed diagnosis and treatment based on risk factors can influence survival in group of patients with systemic primary amyloidosis.
  • [MeSH-major] Amyloidosis / therapy. Hematopoietic Stem Cell Transplantation
  • [MeSH-minor] Adult. Humans. Middle Aged. Myeloablative Agonists / therapeutic use. Risk Factors. Survival Rate

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  • (PMID = 19827724.001).
  • [ISSN] 1426-9686
  • [Journal-full-title] Polski merkuriusz lekarski : organ Polskiego Towarzystwa Lekarskiego
  • [ISO-abbreviation] Pol. Merkur. Lekarski
  • [Language] pol
  • [Publication-type] Editorial; English Abstract
  • [Publication-country] Poland
  • [Chemical-registry-number] 0 / Myeloablative Agonists
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89. Mendenhall WM, Mendenhall CM, Mendenhall NP: Solitary plasmacytoma of bone and soft tissues. Am J Otolaryngol; 2003 Nov-Dec;24(6):395-9
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  • [Title] Solitary plasmacytoma of bone and soft tissues.
  • PURPOSE: To define the optimal treatment and outcomes for patients with solitary plasmacytoma of bone and soft tissue.
  • RESULTS: Solitary plasmacytomas are uncommon and account for less than 5% of plasma cell neoplasms.
  • The optimal treatment is moderate-dose radiotherapy (40-50 Gy) and occasionally surgery.
  • Adjuvant chemotherapy does not improve survival.
  • CONCLUSION: Solitary plasmacytoma is an uncommon neoplasm that often arises in the head and neck.
  • Optimal treatment is moderate-dose radiotherapy.
  • [MeSH-major] Bone Neoplasms / therapy. Plasmacytoma / therapy. Soft Tissue Neoplasms / therapy
  • [MeSH-minor] Female. Humans. Male. Prognosis. Survival Rate. Treatment Outcome

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  • (PMID = 14608572.001).
  • [ISSN] 0196-0709
  • [Journal-full-title] American journal of otolaryngology
  • [ISO-abbreviation] Am J Otolaryngol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 23
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90. Santos ES, Goodman M, Byrnes JJ, Fernandez HF: Thalidomide effects in the post-transplantation setting in patients with multiple myeloma. Hematology; 2004 Feb;9(1):35-9
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  • Thalidomide recently has been proven to have an impact on plasma cell dyscrasia through multiple mechanisms.
  • We report on 12 cases (9 males and 3 females), median age 56 years old (range 41-65 years old) who underwent autologous peripheral stem cell transplantation for multiple myeloma and received thalidomide as maintenance therapy post-transplantation.
  • Patients received various cytoreductive therapies prior to stem cell harvest.
  • Eleven patients were in partial remission (PR) and one in complete remission (CR) on entry into the transplant phase of therapy.
  • One patient failed to tolerate thalidomide due to CNS symptoms and stopped therapy at 12 days.
  • Another patient stopped thalidomide therapy after 71 days, because of severe fatigue secondary to hypothyroidism.
  • Neutropenia, previously reported as an adverse effect in this setting, was not seen to date in our cohort.
  • CONCLUSION: Thalidomide appears to be a safe drug in the post-transplant setting, perhaps adding to the response achieved post-transplant without major toxicity.
  • Longer follow up and future randomized trials will be needed to validate the role of thalidomide and its long-term effect when used as maintenance therapy in the post-transplant setting.
  • [MeSH-major] Multiple Myeloma / therapy. Peripheral Blood Stem Cell Transplantation / methods. Thalidomide / administration & dosage. Thalidomide / toxicity
  • [MeSH-minor] Aged. Antigens, CD34 / analysis. Drug Evaluation. Female. Follow-Up Studies. Graft Survival. Humans. Male. Middle Aged. Transplantation, Autologous. Treatment Outcome

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  • (PMID = 14965866.001).
  • [ISSN] 1024-5332
  • [Journal-full-title] Hematology (Amsterdam, Netherlands)
  • [ISO-abbreviation] Hematology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, CD34; 4Z8R6ORS6L / Thalidomide
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91. Sanchorawala V, Wright DG, Rosenzweig M, Finn KT, Fennessey S, Zeldis JB, Skinner M, Seldin DC: Lenalidomide and dexamethasone in the treatment of AL amyloidosis: results of a phase 2 trial. Blood; 2007 Jan 15;109(2):492-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Lenalidomide and dexamethasone in the treatment of AL amyloidosis: results of a phase 2 trial.
  • In immunoglobulin light chain (AL) amyloidosis, amyloid fibril deposits derived from immunoglobulin light chains produced by a clonal plasma cell dyscrasia accumulate in tissues and damage vital organs.
  • Treatment regimens used in multiple myeloma can be effective in AL amyloidosis; however, patients with this disease often tolerate these regimens poorly because of multisystem organ dysfunction.
  • In this report, we describe results of a phase 2 trial of the use of lenalidomide, as a single agent and in combination with dexamethasone, for the treatment of AL amyloidosis.
  • Thirty-four patients with AL amyloidosis, most with prior therapies, were enrolled in the trial.
  • Fatigue and myelosuppression were the most common treatment-related adverse events (35%), while thromboembolic complications (9%) were the most serious.
  • [MeSH-major] Amyloidosis / drug therapy. Dexamethasone / administration & dosage. Immunoglobulin Light Chains / blood. Thalidomide / analogs & derivatives
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Dose-Response Relationship, Drug. Drug Administration Schedule. Drug Therapy, Combination. Drug Tolerance. Female. Humans. Male. Middle Aged. Predictive Value of Tests. Survival Rate. Treatment Outcome

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  • [CommentIn] Blood. 2010 Sep 16;116(11):1990-1 [20847211.001]
  • (PMID = 16960148.001).
  • [ISSN] 0006-4971
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] eng
  • [Grant] United States / NHLBI NIH HHS / HL / P01 HL68705
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunoglobulin Light Chains; 4Z8R6ORS6L / Thalidomide; 7S5I7G3JQL / Dexamethasone; F0P408N6V4 / lenalidomide
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92. Musolin L, Reyna R, DeBord J: Primary amyloidosis (AL) presenting with nephrotic syndrome: a case report and discussion. W V Med J; 2003 Jan-Feb;99(1):28-30
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Primary amyloidosis (AL) is a rare disorder with only eight cases per million a year.
  • AL is a plasma cell disorder in which neoplastic clonal plasma cells in the bone marrow produce monoclonal immunoglobulin light chains that form protease resistant amyloid fibrils.
  • AL fibrils accumulate within tissues systemically, causing progressive organ impairment to ultimate death.
  • Median survival in AL with treatment is only 17 months depending upon variable prognostic factors.
  • [MeSH-minor] Edema / drug therapy. Edema / etiology. Fatigue / etiology. Female. Humans. Hypercholesterolemia / complications. Middle Aged

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  • (PMID = 12762214.001).
  • [ISSN] 0043-3284
  • [Journal-full-title] The West Virginia medical journal
  • [ISO-abbreviation] W V Med J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 9
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93. Kyle RA, Gertz MA, Lacy MQ, Dispenzieri A: Localized AL amyloidosis of the colon: an unrecognized entity. Amyloid; 2003 Mar;10(1):36-41
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  • Virtually all patients who present with rectal bleeding and amyloid of the colon have evidence of systemic amyloidosis and require therapy.
  • The small subset of patients with amyloidosis localized to the colon must be recognized and treatment avoided.
  • These patients had no evidence of a plasma cell dyscrasia and received no chemotherapy to prevent deposition of amyloid.
  • It is important to recognize this rare subset and avoid treatment with alkylating agents or high-dose therapy followed by autologous stem cell transplantation.
  • Alkylating agent therapy may be associated with myelodysplasia or acute leukemia.
  • In addition, the cost, inconvenience, and morbidity of therapy are avoided by observation.
  • Patients who present with rectal bleeding and a subsequent diagnosis of amyloidosis of the colon likely will be subjected to chemotherapy or transplantation.
  • Such patients must be recognized and treatment avoided if there is no evidence of systemic amyloidosis because they remain stable for many years.

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  • (PMID = 12762141.001).
  • [ISSN] 1350-6129
  • [Journal-full-title] Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis
  • [ISO-abbreviation] Amyloid
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA62242
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
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94. Srkalovic G, Cameron MG, Rybicki L, Deitcher SR, Kattke-Marchant K, Hussein MA: Monoclonal gammopathy of undetermined significance and multiple myeloma are associated with an increased incidence of venothromboembolic disease. Cancer; 2004 Aug 1;101(3):558-66
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  • [Title] Monoclonal gammopathy of undetermined significance and multiple myeloma are associated with an increased incidence of venothromboembolic disease.
  • BACKGROUND: Venous thromboembolic disease (VTD) is a recently recognized complication of thalidomide in combination therapy for patients with multiple myeloma (MM).
  • The authors assessed the frequency of VTD and its risk factors in 612 consecutive patients with plasma cell dyscrasia (PCD) who were evaluated and followed from 1991 to 2001.
  • The type of the treatment regimen did not appear to correlate with the development of VTD.
  • [MeSH-minor] Adult. Age Distribution. Aged. Aged, 80 and over. Case-Control Studies. Comorbidity. Female. Humans. Incidence. Male. Middle Aged. Multivariate Analysis. Paraproteinemias / diagnosis. Paraproteinemias / drug therapy. Paraproteinemias / epidemiology. Probability. Prognosis. Proportional Hazards Models. Retrospective Studies. Risk Assessment. Sex Distribution. Survival Analysis. Thalidomide / adverse effects. Thalidomide / therapeutic use

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  • (PMID = 15274069.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 4Z8R6ORS6L / Thalidomide
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95. Merlini G, Pozzi C: Mechanisms of renal damage in plasma cell dyscrasias: an overview. Contrib Nephrol; 2007;153:66-86
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  • [Title] Mechanisms of renal damage in plasma cell dyscrasias: an overview.
  • The kidney is a target organ in plasma cell dyscrasias.
  • The kidney targeting is due to the concurrence of several factors such as the local catabolism of monoclonal LCs, specific interactions of the monoclonal proteins with tissue and cellular components, and local environmental conditions.
  • Glomerulopathic LCs interact with mesangial cells producing, through two distinct pathways, LC amyloidosis or monoclonal immunoglobulin deposition disease.
  • Tubulopathic LCs damage the proximal tubule causing Fanconi's syndrome or precipitate in the distal tubule determining the cast nephropathy.
  • In patients with known plasma cell dyscrasia, the recognition of these patterns of renal injury should lead to appropriate therapeutic intervention.

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  • (PMID = 17075224.001).
  • [ISSN] 0302-5144
  • [Journal-full-title] Contributions to nephrology
  • [ISO-abbreviation] Contrib Nephrol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Immunoglobulin Light Chains
  • [Number-of-references] 79
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96. Mohri H: Resolution of acquired factor X deficiency with amyloidosis secondary to plasma cell dyscrasia. Am J Hematol; 2004 Dec;77(4):421-2
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  • [Title] Resolution of acquired factor X deficiency with amyloidosis secondary to plasma cell dyscrasia.
  • [MeSH-minor] Aged. Drug Therapy, Combination. Female. Humans. Melphalan / administration & dosage. Melphalan / therapeutic use. Prednisone / administration & dosage. Prednisone / therapeutic use. Treatment Outcome

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  • (PMID = 15551292.001).
  • [ISSN] 0361-8609
  • [Journal-full-title] American journal of hematology
  • [ISO-abbreviation] Am. J. Hematol.
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] United States
  • [Chemical-registry-number] Q41OR9510P / Melphalan; VB0R961HZT / Prednisone
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97. Weisel KC, Böckeler M, Bianchi L, Terracciano LM, Mayer F, Kanz L: Development of rapid light-chain deposition disease in hepatic arteries with severe ischemic cholangitis in a multiple myeloma patient treated with melphalan, prednisone and lenalidomide. Int J Hematol; 2009 Jan;89(1):91-4
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  • [Title] Development of rapid light-chain deposition disease in hepatic arteries with severe ischemic cholangitis in a multiple myeloma patient treated with melphalan, prednisone and lenalidomide.
  • Light-chain deposition disease (LCDD) is a multisystemic disorder associated with plasma cell dyscrasias and multiple myeloma.
  • However, involvement of other tissues such as liver and heart have been described.
  • Here we report a case of severe ischemic cholangitis in a patient with multiple myeloma receiving chemotherapy with melphalan, prednisone, and lenalidomide.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / adverse effects. Cholangitis / etiology. Hepatic Artery / pathology. Immunoglobulin Light Chains. Multiple Myeloma / complications

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  • (PMID = 19101782.001).
  • [ISSN] 1865-3774
  • [Journal-full-title] International journal of hematology
  • [ISO-abbreviation] Int. J. Hematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Immunoglobulin Light Chains; 4Z8R6ORS6L / Thalidomide; F0P408N6V4 / lenalidomide; Q41OR9510P / Melphalan; VB0R961HZT / Prednisone
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98. Yakushijin Y, Sakai I, Takada K, Yasukawa M, Fujita S: [Double B-cell malignancies with simultaneous onset]. Rinsho Ketsueki; 2004 Mar;45(3):218-22
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Double B-cell malignancies with simultaneous onset].
  • We encountered a case of a 59-year-old female who simultaneously contracted a non-Hodgkin lymphoma (NHL) and a plasma cell neoplasm.
  • The patient consulted her physician about her abdominal tumor and anemia in March 1999.
  • She was diagnosed as having NHL (follicular center lymphoma, grade I, stage IIA) after an open tumor biopsy, and treated by cycles of CHOP chemotherapy which resulted in complete remission.
  • However, the patient's abdominal tumor appeared again in March 2000 and she was hospitalized at the Ehime University Hospital.
  • A tumor biopsy was performed laparoscopically at that time.
  • This diagnosis was made after the presence of IgG-lambda M protein when the marrow showed an increase in the number of plasma cells.
  • In a Southern blot analysis which studied the abdominal tumor and the bone marrow cells, each B-cell tumor had a different IgH gene rearrangement pattern.
  • Therefore, this case was diagnosed as an example of the simultaneous existence of two different B-cell tumors.
  • [MeSH-major] Abdominal Neoplasms. Lymphoma, Follicular. Multiple Myeloma. Neoplasms, Multiple Primary
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. B-Lymphocytes / pathology. Fatal Outcome. Female. Gene Rearrangement, B-Lymphocyte, Heavy Chain. Humans. Middle Aged. Neoplasm Recurrence, Local

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  • (PMID = 15103935.001).
  • [ISSN] 0485-1439
  • [Journal-full-title] [Rinshō ketsueki] The Japanese journal of clinical hematology
  • [ISO-abbreviation] Rinsho Ketsueki
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] Japan
  • [Number-of-references] 17
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99. Hayes-Lattin BM, Curtin PT, Fleming WH, Leis JF, Stepan DE, Schubach S, Maziarz RT: Toxic megacolon: a life-threatening complication of high-dose therapy and autologous stem cell transplantation among patients with AL amyloidosis. Bone Marrow Transplant; 2002 Sep;30(5):279-85
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Toxic megacolon: a life-threatening complication of high-dose therapy and autologous stem cell transplantation among patients with AL amyloidosis.
  • AL amyloidosis is a plasma cell disorder in which tissue deposition of immunoglobulin light chains leads to organ dysfunction.
  • Recent reports of high-dose therapy with autologous stem cell transplantation for amyloidosis suggest higher response rates and extended survival compared to those seen with conventional chemotherapy.
  • However, substantial treatment-related toxicity has been observed.
  • [MeSH-major] Amyloidosis / complications. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Hematopoietic Stem Cell Transplantation / adverse effects. Megacolon, Toxic / etiology
  • [MeSH-minor] Humans. Immunoglobulin Light Chains. Male. Middle Aged. Multiple Myeloma / complications. Transplantation Conditioning / adverse effects. Transplantation, Autologous / adverse effects. Treatment Outcome


100. Mele G, Pinna S, Melpignano A, Quarta G: Retrospective case series of three patients with plasma cell leukemia treated with bortezomib-based regimens. Clin Ther; 2010 May;32(5):915-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Retrospective case series of three patients with plasma cell leukemia treated with bortezomib-based regimens.
  • BACKGROUND: Data from the literature have suggested that bortezomib is the only effective agent in the treatment of plasma cell leukemia (PCL), a type of plasma cell dyscrasia characterized by poor prognosis despite conventional chemotherapy including autologous and allogeneic transplantation.
  • These patients were treated with bortezomib variously combined with other drugs outside of clinical trials.
  • Patients 1 and 2 received bortezomib-based regimens (bortezomib 1.3 mg/m2 i.v. once daily on days 1, 4, 8, and 11; dexamethasone 20 mg i.v. once daily on days 1-4 and 8-11; oral cyclophosphamide 50 mg once daily on days 1-21, every 28 days) after 2 previous chemotherapeutic treatments.
  • In all 3 patients, circulating plasma cells persisted.
  • Patients 1 and 2 were not considered candidates for autologous peripheral blood stem cell transplantation (PBSCT) because of their nonresponse to the bortezomib-based regimens and severe deterioration of their clinical conditions (kidney and liver failure) due to disease progression.
  • After further treatment according to the modified protocol for patients with acute lymphatic leukemia (cyclophosphamide 800 mg/m2 i.v. on day 1 and 200 mg/m2 i.v. on days 2-5; vincristine 1.5 mg/m2 i.v. once daily on days 1, 8, and 15; doxorubicin 40 mg/m2 i.v. on day 1; methotrexate 1200 mg/m2/h i.v.
  • The patient died 5 months later because of disease progression.
  • CONCLUSION: These 3 patients with primary or secondary PCL who received a bortezomib-based regimen as rescue medication did not respond to treatment.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Boronic Acids / administration & dosage. Leukemia, Plasma Cell / drug therapy. Pyrazines / administration & dosage

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  • [Copyright] Copyright 2010 Excerpta Medica Inc. All rights reserved.
  • (PMID = 20685499.001).
  • [ISSN] 1879-114X
  • [Journal-full-title] Clinical therapeutics
  • [ISO-abbreviation] Clin Ther
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Boronic Acids; 0 / Pyrazines; 69G8BD63PP / Bortezomib
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