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1. Auwerda JJ, Sonneveld P, de Maat MP, Leebeek FW: Prothrombotic coagulation abnormalities in patients with paraprotein-producing B-cell disorders. Clin Lymphoma Myeloma; 2007 Jul;7(7):462-6
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  • [Title] Prothrombotic coagulation abnormalities in patients with paraprotein-producing B-cell disorders.
  • In patients with MM, plasma levels of several prothrombotic coagulation factors are increased, and this can contribute to the prothrombotic state of these patients.
  • Recently, an increased thrombosis risk has also been described for other plasma cell disorders (PCDs), such as monoclonal gammopathy of uncertain significance (MGUS) and systemic amyloidosis.
  • The aim of this study was to analyze prothrombotic coagulation disorders in patients with paraprotein-producing B-cell disorders, such as MGUS, systemic amyloidosis, Waldenström's macroglobulinemia, and MM.
  • PATIENTS AND METHODS: An increase in factor VIII and von Willebrand factor was observed in patients with MGUS and systemic amyloidosis that was similar to increases seen in patients with untreated MM.

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  • (PMID = 17875234.001).
  • [ISSN] 1557-9190
  • [Journal-full-title] Clinical lymphoma & myeloma
  • [ISO-abbreviation] Clin Lymphoma Myeloma
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Blood Coagulation Factors; 0 / Paraproteins
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2. Garces-Sanchez M, Dyck PJ, Kyle RA, Zeldenrust S, Wu Y, Ladha SS, Klein CJ: Antibodies to myelin-associated glycoprotein (anti-Mag) in IgM amyloidosis may influence expression of neuropathy in rare patients. Muscle Nerve; 2008 Apr;37(4):490-5
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  • Anti-MAG and the cross-reacted sulfoglucuronyl paragloboside antibodies (SGPG) were studied in 46 patients with IgM amyloidosis (21 with polyneuropathy), and 21 matched IgM MGUS (monoclonal gammopathies of undetermined significance) controls without neuropathy.
  • Low levels of anti-MAG antibodies were found in 12 of 21 IgM MGUS controls without neuropathy (mean follow-up, 11 years).
  • We conclude that finding serum anti-MAG antibodies does not exclude the diagnosis of primary amyloidosis.

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  • (PMID = 18236455.001).
  • [ISSN] 0148-639X
  • [Journal-full-title] Muscle & nerve
  • [ISO-abbreviation] Muscle Nerve
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA 62242; United States / NINDS NIH HHS / NS / NS 36797
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Autoantibodies; 0 / Globosides; 0 / Immunoglobulin M; 0 / Myelin-Associated Glycoprotein; 0 / sulfate-3-glucuronyl paragloboside
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3. Au GG, Lincz LF, Enno A, Shafren DR: Oncolytic Coxsackievirus A21 as a novel therapy for multiple myeloma. Br J Haematol; 2007 Apr;137(2):133-41
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  • [Title] Oncolytic Coxsackievirus A21 as a novel therapy for multiple myeloma.
  • This study assessed the capacity of Coxsackievirus A21 (CVA21) to target and destroy multiple myeloma (MM) and precursor aberrant plasma cells in vitro using established MM cell lines and 15 patient bone marrow (BM) biopsies [n = 10 MM and five monoclonal gammopathy of undetermined significance (MGUS)].
  • Cell surface analysis revealed that all tumour cells lines expressed high levels of intercellular adhesion molecule-1 (ICAM-1) and decay-accelerating factor (DAF), the receptor molecules to which CVA21 can bind, leading to subsequent cell-entry and infection.
  • MM cell lines were remarkably susceptible to CVA21 lytic infection, producing 100-1000-fold increases in viral progeny within 24 h.
  • Furthermore, challenge of patient BM biopsies with CVA21 for 48 h resulted in specific purging of up to 98.7% of CD138+ plasma cells, with no significant decrease in progenitor cell function.
  • Data generated in this study suggests that CVA21 virotherapy may have potential applications as a systemic anti-tumour agent for MM, or in the ex vivo purging of malignant plasma cells prior to autologous stem cell transplantation.
  • [MeSH-minor] Antigens, CD55 / metabolism. Bone Marrow Cells / pathology. Bone Marrow Purging / methods. Cell Death. Coculture Techniques. Colony-Forming Units Assay. Humans. Intercellular Adhesion Molecule-1 / metabolism. Leukocytes, Mononuclear / pathology. Leukocytes, Mononuclear / virology. Neoplasm Proteins / metabolism. Tumor Cells, Cultured. Virus Replication

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  • (PMID = 17391493.001).
  • [ISSN] 0007-1048
  • [Journal-full-title] British journal of haematology
  • [ISO-abbreviation] Br. J. Haematol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, CD55; 0 / Neoplasm Proteins; 126547-89-5 / Intercellular Adhesion Molecule-1
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4. Okura T, Miyoshi K, Nagao T, Jotoku M, Enomoto D, Irita J, Kurata M, Higaki J: Light chain deposition disease developing 15 years following the diagnosis of monoclonal gammopathy of undetermined significance. Intern Med; 2009;48(2):101-4
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  • [Title] Light chain deposition disease developing 15 years following the diagnosis of monoclonal gammopathy of undetermined significance.
  • Fifteen years previously she had been diagnosed with monoclonal gammopathy of undetermined significance (MGUS).
  • Her renal biopsy specimen revealed thickened basement membrane, mesangial cell proliferation and an increase in the mesangial matrix.
  • These findings confirmed a diagnosis of light chain deposit disease (LCDD) with MGUS.
  • The development of LCDD in patients with MGUS for fifteen years is very rare.

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  • (PMID = 19145054.001).
  • [ISSN] 1349-7235
  • [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 / Immunoglobulin A; 0 / Immunoglobulin kappa-Chains
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5. Aline-Fardin A, Rifle G, Martin L, Justrabo E, Bour JB, D'Athis P, Tanter Y, Mousson C: Recurent and de novo membranous glomerulopathy after kidney transplantation. Transplant Proc; 2009 Mar;41(2):669-71
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  • [Title] Recurent and de novo membranous glomerulopathy after kidney transplantation.
  • The aim of this study was to compare the clinical characteristics of recurrent and de novo membranous glomerulopathy (MG) among a cohort of 614 recipients transplanted between 1989 and 2006.
  • The diagnosis was established on protocol biopsies performed 1, 2, 4, or 8 years after transplantation or because of proteinuria/nephrotic syndrome and/or an increased serum creatinine level.
  • HCV infection, cryoglobulinemia, monoclonal gammopathy, skin cancers, Kaposi sarcoma, diabetes mellitus, anti-HLA antibodies, and graft survival were not significantly different between the groups.
  • Seventeen MG were diagnosed in 15 patients (2.45% of the whole group), including 6 recurrent MG (35%) and 11 de novo MG (75%).
  • Recurrent MG occurred earlier than de novo MG (15.58 +/- 19.13 vs 49.27 +/- 32.71 months).
  • Recipients with de novo MG were more frequently infected with HCV, which seemed to be the main etiologic factor for de novo MG, and may be linked to a Th2 polarization of the immune response.

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  • (PMID = 19328952.001).
  • [ISSN] 0041-1345
  • [Journal-full-title] Transplantation proceedings
  • [ISO-abbreviation] Transplant. Proc.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunosuppressive Agents
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6. Dupont SA, Dispenzieri A, Mauermann ML, Rabinstein AA, Brown RD Jr: Cerebral infarction in POEMS syndrome: incidence, risk factors, and imaging characteristics. Neurology; 2009 Oct 20;73(16):1308-12
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  • OBJECTIVES: To determine the risk factors and incidence of cerebral infarction associated with POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome.
  • METHODS: The Mayo Clinic dysproteinemia database was queried to identify patients with coded diagnosis of POEMS syndrome.
  • Evidence of plasma cell proliferation within the bone marrow and elevated serum platelet count led to increased risk of cerebral infarction in this population.
  • [MeSH-minor] Adult. Aged. Bone Marrow / pathology. Bone Marrow / physiopathology. Brain / pathology. Brain / radiography. Cell Proliferation. Cohort Studies. Databases, Factual. Female. Humans. Incidence. Magnetic Resonance Imaging. Male. Middle Aged. Plasma Cells / pathology. Plasma Cells / physiology. Platelet Count. Retrospective Studies. Risk Factors. Tomography, X-Ray Computed

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  • (PMID = 19841383.001).
  • [ISSN] 1526-632X
  • [Journal-full-title] Neurology
  • [ISO-abbreviation] Neurology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC2764416
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7. Terrier B, Lacroix C, Guillevin L, Hatron PY, Dhote R, Maillot F, Diot E, Sarrot-Reynauld F, Sordet C, Dubourg O, Meyer L, Mariette X, Gottenberg JE, Club Rhumatismes et Inflammation: Diagnostic and prognostic relevance of neuromuscular biopsy in primary Sjögren's syndrome-related neuropathy. Arthritis Rheum; 2007 Dec 15;57(8):1520-9
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  • RESULTS: Patients with vasculitis (lymphocytic [n = 8] or necrotizing [n = 14]) had a higher prevalence of acute-onset neuropathy, multiple mononeuropathy, sensorimotor involvement, vascular purpura, general symptoms, increased C-reactive protein level, positivity for rheumatoid factor, hypocomplementemia, and monoclonal gammopathy compared with those without vasculitis (n = 18).
  • Regarding clinical evolution, the results of NMB (P < 0.0001), in particular the presence of necrotizing vasculitis (P < 0.001), an acute neuropathy onset (P < 0.0001), general symptoms (P < 0.0001), multiple mononeuropathy (P = 0.0007), presence of sensorimotor involvement (P = 0.002), and increased C-reactive protein level (P = 0.008), were significantly associated with a better outcome in univariate analysis.
  • [MeSH-major] Muscle, Skeletal / pathology. Peripheral Nervous System Diseases / diagnosis. Peripheral Nervous System Diseases / etiology. Sjogren's Syndrome / complications. Sural Nerve / pathology
  • [MeSH-minor] Adrenal Cortex Hormones / therapeutic use. Adult. Aged. Biopsy. C-Reactive Protein / metabolism. Disease Progression. Female. Humans. Immunosuppressive Agents / therapeutic use. Male. Middle Aged. Multivariate Analysis. Prognosis. Retrospective Studies. Vasculitis / diagnosis. Vasculitis / etiology. Vasculitis / pathology

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  • (PMID = 18050172.001).
  • [ISSN] 0004-3591
  • [Journal-full-title] Arthritis and rheumatism
  • [ISO-abbreviation] Arthritis Rheum.
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Adrenal Cortex Hormones; 0 / Immunosuppressive Agents; 9007-41-4 / C-Reactive Protein
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8. Randi ML, Ruzzon E, Tezza F, Tezza F, Pacquola E, Fabris F: Monoclonal gammopathy in human leishmaniasis. Neth J Med; 2006 Feb;64(2):50-1
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  • [Title] Monoclonal gammopathy in human leishmaniasis.
  • A 64-year-old female with IgGk monoclonal components (total 45 g/l) and 30% abnormal plasma cells and plasmoblasts in bone marrow is reported.
  • After the identification of leishmania in the bone marrow, liposomal amphotericin B was used and a progressive resolution of the gammopathy was documented.
  • [MeSH-major] Amphotericin B / therapeutic use. Antiprotozoal Agents / therapeutic use. Bone Marrow Diseases / parasitology. Leishmaniasis, Visceral / diagnosis
  • [MeSH-minor] Diagnosis, Differential. Female. Humans. Immunoglobulin G / blood. Liposomes. Middle Aged. Paraproteinemias / blood

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  • (PMID = 16517989.001).
  • [ISSN] 0300-2977
  • [Journal-full-title] The Netherlands journal of medicine
  • [ISO-abbreviation] Neth J Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Antiprotozoal Agents; 0 / Immunoglobulin G; 0 / Liposomes; 7XU7A7DROE / Amphotericin B
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9. Lannert H, Able T, Becker S, Sommer M, Braun M, Stadtherr P, Ho AD: Optimizing BM harvesting from normal adult donors. Bone Marrow Transplant; 2008 Oct;42(7):443-7
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  • The experience at a single institution of BM harvesting (BMH) in general anesthetic for allogeneic transplantation from 49 healthy adult donors since March 2002 is presented in detail, together with an analysis of all the donor complications.
  • BMH accomplished by trained personal is a safe procedure for healthy adult donors on an outpatient basis as standard in our collection center.
  • [MeSH-major] Bone Marrow Cells / cytology. Bone Marrow Cells / physiology. Hematopoietic Stem Cell Transplantation / methods. Tissue and Organ Harvesting / methods

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  • (PMID = 18622419.001).
  • [ISSN] 0268-3369
  • [Journal-full-title] Bone marrow transplantation
  • [ISO-abbreviation] Bone Marrow Transplant.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Antigens, CD34; 0 / Hemoglobins; 143011-72-7 / Granulocyte Colony-Stimulating Factor
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10. Katzmann JA, Abraham RS, Dispenzieri A, Lust JA, Kyle RA: Diagnostic performance of quantitative kappa and lambda free light chain assays in clinical practice. Clin Chem; 2005 May;51(5):878-81
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  • BACKGROUND: The quantitative assay for free light chains (FLCs) is a recently introduced commercial test reported to be sensitive and specific for detecting FLC diseases such as primary systemic amyloidosis (AL), light chain deposition disease (LCDD), nonsecretory multiple myeloma (NSMM), and light chain multiple myeloma.
  • The majority of these patients (88%) had bone marrow-derived monoclonal plasma cell disorders (PCDs).
  • The 121 patients who did not have monoclonal gammopathy all had FLC kappa/lambda ratios within the range of values obtained for a reference population in our laboratory.
  • Among the patients with monoclonal gammopathies were patients with multiple myeloma (330), AL (269), monoclonal gammopathy of undetermined significance (114), smoldering multiple myeloma (72), plasmacytoma (22), NSMM (20), macroglobulinemia (9), LCDD (7), and a variety of other PCDs.
  • Among the 110 AL patients who had not been previously treated and who had a FLC assay performed within 120 days of diagnosis, the FLC kappa/lambda ratio was positive in 91% compared with 69% for serum immunofixation electrophoresis (IFE) and 83% for urine IFE.
  • [MeSH-major] Amyloidosis / diagnosis. Immunoglobulin kappa-Chains / blood. Immunoglobulin lambda-Chains / blood. Paraproteinemias / diagnosis
  • [MeSH-minor] Clinical Laboratory Information Systems. Humans. Laboratories, Hospital. Medical Records Systems, Computerized. Multiple Myeloma / diagnosis. Plasmacytoma / diagnosis. Waldenstrom Macroglobulinemia / diagnosis

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  • [CommentIn] Clin Chem. 2005 May;51(5):805-7 [15855664.001]
  • (PMID = 15774572.001).
  • [ISSN] 0009-9147
  • [Journal-full-title] Clinical chemistry
  • [ISO-abbreviation] Clin. Chem.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA62242
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunoglobulin kappa-Chains; 0 / Immunoglobulin lambda-Chains
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11. Cook L, Macdonald DH: Management of paraproteinaemia. Postgrad Med J; 2007 Apr;83(978):217-23
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  • A paraprotein is a monoclonal immunoglobulin or light chain present in the blood or urine; it is produced by a clonal population of mature B cells, most commonly plasma cells.
  • In individuals aged >50 years the incidence of a paraprotein is 3.2%.
  • Plasma cell disorders can be considered as a spectrum of conditions ranging from monoclonal gammopathy of undetermined significance (MGUS), through asymptomatic, to symptomatic myeloma.
  • MGUS is defined by a low level of paraprotein <30 g/l, bone marrow plasma cells <10% and the absence of myeloma related organ or tissue damage (predominantly renal, skeletal or bone marrow impairment.
  • ) MGUS requires no therapy and the overall risk of progression to myeloma is 1% per year.
  • Myeloma remains incurable with a median survival of 3-4 years; autologous stem cell transplant can prolong survival, if appropriate.
  • [MeSH-minor] Anemia / etiology. Diagnosis, Differential. Diphosphonates / therapeutic use. Humans. Hypercalcemia / therapy. Infection / etiology. Kidney Diseases / etiology. Multiple Myeloma / diagnosis. Pain / prevention & control. Prognosis. Referral and Consultation

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  • (PMID = 17403946.001).
  • [ISSN] 1469-0756
  • [Journal-full-title] Postgraduate medical journal
  • [ISO-abbreviation] Postgrad Med J
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Diphosphonates
  • [Number-of-references] 37
  • [Other-IDs] NLM/ PMC2600027
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12. Liebisch P, Eppinger S, Schöpflin C, Stehle G, Munzert G, Döhner H, Schmid M: CD44v6, a target for novel antibody treatment approaches, is frequently expressed in multiple myeloma and associated with deletion of chromosome arm 13q. Haematologica; 2005 Apr;90(4):489-93
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  • BACKGROUND AND OBJECTIVES: Despite recent advances in the treatment of multiple myeloma (MM), this disease remains incurable in the majority of patients.
  • To investigate the applicability of this compound to clonal plasma cell disorders, we analyzed CD44v6 expression on malignant plasma cells from patients with multiple myeloma.
  • DESIGN AND METHODS: Bone marrow samples from 57 patients with monoclonal gammopathy of undetermined significance (MGUS), MM, and plasma cell leukemia (PCL) were examined for CD44v6 expression by using flow cytometry (FACS) analysis.
  • RESULTS: In only 1 of 16 cases (6%) with MGUS and 1 out of 6 cases (17%) with stage I MM were plasma cells CD44v6 positive.
  • These results suggest that this epitope is a potential new target for monoclonal antibodies such as bivatuzumab mertansine.
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Antibodies, Monoclonal / therapeutic use. Antineoplastic Agents / therapeutic use. Female. Humans. Leukemia, Plasma Cell / drug therapy. Leukemia, Plasma Cell / genetics. Leukemia, Plasma Cell / immunology. Male. Middle Aged. Paraproteinemias / drug therapy. Paraproteinemias / genetics. Paraproteinemias / immunology. Tumor Burden / immunology


13. 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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  • [Title] [Neuropathy associated with monoclonal gammopathy (dysglobulinemia)].
  • [Transliterated title] Neuropathies associées aux gammapathies monoclonales (dysglobulinémies).
  • Neurological complications of monoclonal gammopathy, or dysglobulinemia, are typically affecting the peripheral nerve.
  • 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.

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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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14. Müntener T, Rüfenacht S, Di Palma S, Hartmeier G, Welle M: Scleromyxedema-like syndrome with systemic involvement in a cat. Vet Pathol; 2010 Mar;47(2):346-50
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  • Scleromyxedema--the generalized form of lichen myxedematosus, a primary mucinosis--is a rare disease in human patients.
  • It is characterized by dermal mucin deposits, increased numbers of fibroblasts, and variable fibrosis in the absence of thyroid disease.
  • It is accompanied in 80% of cases by a monoclonal gammopathy.
  • This is the first report of a scleromyxedema-like syndrome in a cat, which had a substantial deposition of mucin in the dermis of the head and paws with a mild gammaglobulinemia of 2.25 g/dl (reference range, 1.39-2.22 g/dl).

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  • (PMID = 20110223.001).
  • [ISSN] 1544-2217
  • [Journal-full-title] Veterinary pathology
  • [ISO-abbreviation] Vet. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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16. Lee YS, Kim SY, Kwon CW, Song HG, Lee YK, Kim HJ, Zang DY: Two cases of systemic capillary leak syndrome that were treated with pentastarch. Korean J Intern Med; 2007 Jun;22(2):130-2
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  • Systemic capillary leak syndrome (SCLS) is a condition that's caused by the shift of fluid and protein from the intravascular space to the interstitial space as a result of repetitive episodes of capillary hyperpermeability.
  • The pathogenesis of SCLS is still unclear, but there's recently been a report showing this syndrome in association with monoclonal gammopathy.
  • This syndrome can be a fatal disease because cardiovascular collapse can occur in the initial capillary leak phase.
  • Considering that this disease is self-limiting, conservative treatment in the acute phase is believed to be very important.
  • [MeSH-major] Capillary Leak Syndrome / drug therapy. Hydroxyethyl Starch Derivatives / therapeutic use. Plasma Substitutes / therapeutic use

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  • [Cites] J Intern Med. 2000 Jun;247(6):731-5 [10886496.001]
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  • (PMID = 17616032.001).
  • [ISSN] 1226-3303
  • [Journal-full-title] The Korean journal of internal medicine
  • [ISO-abbreviation] Korean J. Intern. Med.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Korea (South)
  • [Chemical-registry-number] 0 / Hydroxyethyl Starch Derivatives; 0 / Plasma Substitutes
  • [Other-IDs] NLM/ PMC2687610
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17. McMaster ML, Csako G: Protein electrophoresis, immunoelectrophoresis and immunofixation electrophoresis as predictors for high-risk phenotype in familial Waldenström macroglobulinemia. Int J Cancer; 2008 Mar 1;122(5):1183-8
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  • Protein electrophoresis is used for the detection, evaluation and follow-up of monoclonal gammopathy (MG) conditions such as Waldenström macroglobulinemia (WM).
  • Immunofixation electrophoresis (IFE) is currently the most common method for isotyping of monoclonal gammopathy because of its superior sensitivity relative to immunoelectrophoresis (IEP).
  • We designed a study to evaluate the clinicobiological relevance of small monoclonal bands detected by serum protein electrophoresis, IEP, and IFE.
  • Serum protein electrophoresis, IEP, and IFE were used to evaluate possible monoclonal gammopathy in 46 members (29 relatives and 17 nonbloodline spouses) from 3 families with multiple cases of WM.
  • IFE identified small monoclonal bands initially missed by IEP in 5 individuals (2 blood relatives, 3 spouses) among 46 study participants.
  • Twenty-three individuals, including the 2 blood relatives and 2 of 3 spouses with monoclonal gammopathy, were then followed for a median of 17 years (range, 13-25).
  • The monoclonal gammopathy progressed in the 2 relatives but disappeared in the spouses, and new IgM MG developed in 2 additional relatives with a prior history of IgM polyclonal gammopathy.
  • Small monoclonal bands detected by IFE in a familial context may be biologically meaningful, both as phenotypic biomarkers and possibly as predictors of high risk for WM.
  • [MeSH-major] Blood Protein Electrophoresis. Electrophoresis, Agar Gel. Immunoelectrophoresis. Waldenstrom Macroglobulinemia / blood. Waldenstrom Macroglobulinemia / diagnosis

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 17990319.001).
  • [ISSN] 1097-0215
  • [Journal-full-title] International journal of cancer
  • [ISO-abbreviation] Int. J. Cancer
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunoglobulin M
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18. Miyazaki D, Yazaki M, Gono T, Kametani F, Tsuchiya A, Matsuda M, Takenaka Y, Hosh Y 2nd, Ikeda S: AH amyloidosis associated with an immunoglobulin heavy chain variable region (VH1) fragment: a case report. Amyloid; 2008 Jun;15(2):125-8
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  • We report a 67-year-old male patient who suffered from nephrotic syndrome and progressive renal dysfunction with monoclonal gammopathy (IgMkappa).
  • Biochemical analysis of the deposited amyloid fibrils in gastroduodenal mucosa revealed that the amyloid fibrils were composed of an immunoglobulin heavy chain variable region (VH) fragment belonging to the VH1 subgroup, and a diagnosis of AH amyloidosis was made.
  • In our institute, three patients with AH amyloidosis including the present one have been identified during the past 2 years, so AH amyloidosis seems to be by no means a rare disorder.

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  • (PMID = 18484339.001).
  • [ISSN] 1744-2818
  • [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; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Amyloid; 0 / Immunoglobulin Heavy Chains; 0 / Immunoglobulin Variable Region
  • [Number-of-references] 11
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19. Bouvard B, Royer M, Chappard D, Audran M, Hoppé E, Legrand E: Monoclonal gammopathy of undetermined significance, multiple myeloma, and osteoporosis. Joint Bone Spine; 2010 Mar;77(2):120-4
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  • [Title] Monoclonal gammopathy of undetermined significance, multiple myeloma, and osteoporosis.
  • The finding of monoclonal gammopathy of undetermined significance (MGUS) is not infrequent during an evaluation for osteoporosis or a fracture.
  • In most cases, the diagnosis is MGUS, whose prevalence increases with age.
  • Although the impact of MGUS on bone mineral density, bone remodeling, and the fracture risk remains unclear, this asymptomatic hematological disorder may constitute a risk factor for osteoporosis.
  • Furthermore, each year, 1% of patients with MGUS progress to multiple myeloma, a disease whose pathophysiology and association with bone loss and pathological fractures are increasingly well understood.
  • Here, we discuss the pathophysiology of myeloma and MGUS and their impact in terms of bone mineral density, osteoporotic fractures, and bone turnover markers.
  • [MeSH-minor] Disease Progression. Fractures, Bone / epidemiology. Fractures, Bone / physiopathology. Humans. Prevalence

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  • [Copyright] Copyright 2009 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.
  • (PMID = 20097594.001).
  • [ISSN] 1778-7254
  • [Journal-full-title] Joint, bone, spine : revue du rhumatisme
  • [ISO-abbreviation] Joint Bone Spine
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] France
  • [Number-of-references] 46
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20. Racanelli V, Leone P, Frassanito MA, Brunetti C, Perosa F, Ferrone S, Dammacco F: Alterations in the antigen processing-presenting machinery of transformed plasma cells are associated with reduced recognition by CD8+ T cells and characterize the progression of MGUS to multiple myeloma. Blood; 2010 Feb 11;115(6):1185-93
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  • [Title] Alterations in the antigen processing-presenting machinery of transformed plasma cells are associated with reduced recognition by CD8+ T cells and characterize the progression of MGUS to multiple myeloma.
  • We hypothesized that progression of monoclonal gammopathy of undetermined significance (MGUS) to multiple myeloma (MM) reflects the escape of transformed plasma cells from T-cell recognition because of impaired antigen processing-presenting machinery (APM).
  • We studied plasma cells and CD8(+) T cells from bone marrow of 20 MGUS patients, 20 MM patients, and 10 control patients.
  • Immunofluorescence and flow cytometry revealed significantly different patterns of APM component expression in plasma cells from the 3 groups.
  • MGUS samples had intermediate percentages of stained cells but molecular equivalents of soluble fluorochrome similar to control patients.
  • Cytotoxicity assays demonstrated that MGUS CD8(+) T cells lysed autologous transformed plasma cells more than MM CD8(+) T cells did.
  • MGUS progression correlated directly with calnexin, calreticulin, and tapasin and indirectly with delta, LMP2, and LMP10 expression levels; MM disease status did not correlate with APM levels.
  • APM changes may allow transformed plasma cells to elude immunesurveillance in the MGUS-MM pathogenetic sequence.

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  • (PMID = 20008301.001).
  • [ISSN] 1528-0020
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA113861; United States / NCI NIH HHS / CA / R01 CA110249; United States / NCI NIH HHS / CA / CA109688,; United States / NCI NIH HHS / CA / CA110249; United States / NCI NIH HHS / CA / P01 CA109688; United States / NCI NIH HHS / CA / R01 CA113861
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Interleukin-6; 0 / RNA, Messenger; 82115-62-6 / Interferon-gamma
  • [Other-IDs] NLM/ PMC2826230
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21. Ocio EM, Mateo G, Vidriales B, Lopez-Berges MC, Garcia-Sanz R, Hernandez JM, Orfao A, San Miguel JF: Cell cycle analysis of Waldenstrom's macroglobulinemia. Clin Lymphoma; 2005 Mar;5(4):250-2
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  • [Title] Cell cycle analysis of Waldenstrom's macroglobulinemia.
  • Little is known about the DNA cell content and cell cycle characteristics of immunoglobulin (Ig) M monoclonal gammopathies.
  • The autonomous clone appears to be rather heterogeneous, from mature B lymphocytes to plasma cells (PCs).
  • We have evaluated the DNA cell content of 27 patients with IgM monoclonal gammopathies: 18 of them had Waldenstrom's macroglobulinemia (WM), and 9 were diagnosed with IgM-monoclonal gammopathy of undetermined significance (MGUS).
  • To specifically analyze the cell cycle of the B lymphocyte and PC populations, we used a flow-cytometric double-staining technique with CD19/CD20/CD22 propidium iodide for B lymphocytes and CD38/CD138 propidium iodide for PCs.
  • In 26 of 27 patients, both subsets of tumor cells (B lymphocyte and PC) showed a diploid DNA cell content (DNA index, 1).
  • No differences were observed when comparing the proliferative activity of WM with that of IgM MGUS (median, 1.7% vs. 2.2%, respectively).
  • Cell cycle characteristics of PCs were simultaneously evaluated in 9 patients, with 1.8% cells in S phase or G2/M phase.
  • In summary, the cell cycle analysis showed that IgM monoclonal gammopathies are low-proliferative disorders, with a DNA ploidy pattern (diploid) clearly different from that of multiple myeloma.
  • [MeSH-major] Cell Cycle. DNA / analysis. Immunoglobulin M / immunology. Ploidies. Waldenstrom Macroglobulinemia / genetics. Waldenstrom Macroglobulinemia / physiopathology
  • [MeSH-minor] Cell Proliferation. Flow Cytometry. Humans. Multiple Myeloma / genetics. Multiple Myeloma / physiopathology. Plasma Cells / physiology

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  • (PMID = 15794858.001).
  • [ISSN] 1526-9655
  • [Journal-full-title] Clinical lymphoma
  • [ISO-abbreviation] Clin Lymphoma
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunoglobulin M; 9007-49-2 / DNA
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22. Eby C: Pathogenesis and management of bleeding and thrombosis in plasma cell dyscrasias. Br J Haematol; 2009 Apr;145(2):151-63
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  • [Title] Pathogenesis and management of bleeding and thrombosis in plasma cell dyscrasias.
  • Unexpectedly high rates of venous thromboembolic events (VTE) concurrent with the introduction of highly effective immune modulating drugs thalidomide and lenolidomide for treatment of multiple myeloma have focused attention on the incidence and underlying pathophysiology of VTE in patients with plasma cell dyscrasias, and on thromboprophylaxis approaches.
  • While bleeding complications are relatively uncommon in patients with lymphoproliferative disorders, acquired von Willebrand syndrome, typically occurring in patients with monoclonal gammopathy of unknown significance, and acquired coagulopathies associated with primary amyloidosis can present with haemorrhagic complications and both are challenging to manage.
  • This review highlights these important haemostasis-related complications of plasma cell dyscrasias and provides an overview of other uncommon bleeding and thrombotic events that can affect diagnostic and therapeutic management of clonal plasma cell disorders.

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  • (PMID = 19210509.001).
  • [ISSN] 1365-2141
  • [Journal-full-title] British journal of haematology
  • [ISO-abbreviation] Br. J. Haematol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Boronic Acids; 0 / Immunosuppressive Agents; 0 / Protease Inhibitors; 0 / Pyrazines; 4Z8R6ORS6L / Thalidomide; 69G8BD63PP / Bortezomib
  • [Number-of-references] 145
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23. Pérez-Persona E, Vidriales MB, Mateo G, García-Sanz R, Mateos MV, de Coca AG, Galende J, Martín-Nuñez G, Alonso JM, de Las Heras N, Hernández JM, Martín A, López-Berges C, Orfao A, San Miguel JF: New criteria to identify risk of progression in monoclonal gammopathy of uncertain significance and smoldering multiple myeloma based on multiparameter flow cytometry analysis of bone marrow plasma cells. Blood; 2007 Oct 1;110(7):2586-92
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  • [Title] New criteria to identify risk of progression in monoclonal gammopathy of uncertain significance and smoldering multiple myeloma based on multiparameter flow cytometry analysis of bone marrow plasma cells.
  • Monoclonal gammopathy of uncertain significance (MGUS) and smoldering multiple myeloma (SMM) are plasma cell disorders with a risk of progression of approximately 1% and 10% per year, respectively.
  • We have previously shown that the proportion of bone marrow (BM) aberrant plasma cells (aPCs) within the BMPC compartment (aPC/BMPC) as assessed by flow cytometry (FC) contributes to differential diagnosis between MGUS and multiple myloma (MM).
  • The goal of the present study was to investigate this parameter as a marker for risk of progression in MGUS (n = 407) and SMM (n = 93).
  • Patients with a marked predominance of aPCs/BMPC (> or = 95%) at diagnosis displayed a significantly higher risk of progression both in MGUS and SMM (P< .001).
  • Multivariate analysis for progression-free survival (PFS) selected the percentage aPC/BMPC (> or = 95%) as the most important independent variable, together with DNA aneuploidy and immunoparesis, for MGUS and SMM, respectively.
  • Using these independent variables, we have identified 3 risk categories in MGUS (PFS at 5 years of 2%, 10%, and 46%, respectively; P< .001) and SMM patients (PFS at 5 years of 4%, 46%, and 72%, respectively; P < .001).
  • Our results show that multiparameter FC evaluation of BMPC at diagnosis is a valuable tool that could help to individualize the follow-up strategy for MGUS and SMM patients.
  • [MeSH-major] Bone Marrow Cells / pathology. Multiple Myeloma / classification. Multiple Myeloma / pathology. Paraproteinemias / classification. Paraproteinemias / pathology. Plasma Cells / pathology
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Disease Progression. Female. Flow Cytometry. Humans. Male. Middle Aged. Phenotype. Risk Factors


24. Lendvai N, Gnjatic S, Ritter E, Mangone M, Austin W, Reyner K, Jayabalan D, Niesvizky R, Jagannath S, Bhardwaj N, Chen-Kiang S, Old LJ, Cho HJ: Cellular immune responses against CT7 (MAGE-C1) and humoral responses against other cancer-testis antigens in multiple myeloma patients. Cancer Immun; 2010 Jan 29;10:4
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  • The type I melanoma antigen gene (MAGE) proteins CT7 (MAGE-C1) and MAGE-A3 are commonly expressed in multiple myeloma (MM), and their expression correlates with increased plasma cell proliferation and poor clinical outcome.
  • We analyzed cellular and humoral immune responses against CTAgs in patients with plasma cell dyscrasias: MM, monoclonal gammopathy of undetermined significance (MGUS), and Waldenström's macroglobulinemia (WM).
  • [MeSH-major] Antigens, Neoplasm / immunology. Immunity, Cellular. Immunity, Humoral. Multiple Myeloma / immunology. Neoplasm Proteins / immunology
  • [MeSH-minor] Aged. Cell Separation. Enzyme-Linked Immunosorbent Assay. Female. Flow Cytometry. Fluorescent Antibody Technique. Humans. Male. Middle Aged. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 20108890.001).
  • [ISSN] 1424-9634
  • [Journal-full-title] Cancer immunity
  • [ISO-abbreviation] Cancer Immun.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / K01 CA115917; United States / NCI NIH HHS / CA / K01 CA115917-04; United States / NCI NIH HHS / CA / NCI K01-CA115917
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, Neoplasm; 0 / MAGEA3 protein, human; 0 / MAGEC1 protein, human; 0 / Neoplasm Proteins
  • [Other-IDs] NLM/ NIHMS227264; NLM/ PMC2926649
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25. Chen L, Wang S, Zhou Y, Wu X, Entin I, Epstein J, Yaccoby S, Xiong W, Barlogie B, Shaughnessy JD Jr, Zhan F: Identification of early growth response protein 1 (EGR-1) as a novel target for JUN-induced apoptosis in multiple myeloma. Blood; 2010 Jan 7;115(1):61-70
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  • [Title] Identification of early growth response protein 1 (EGR-1) as a novel target for JUN-induced apoptosis in multiple myeloma.
  • Tumor-bone marrow microenvironment interactions in multiple myeloma (MM) are documented to play crucial roles in plasma-cell growth/survival.
  • In vitro coculture of MM cells with osteoclasts supported cell survival and significantly down-regulated JUN expression.
  • JUN expression in myeloma cells from late-stage and high-risk MM was significantly lower than in plasma cells from healthy donors, monoclonal gammopathy of undetermined significance, smoldering MM, and low-risk MM; patients with low-JUN-expressing MM cells had earlier disease-related deaths.
  • JUN overexpression in MM cells induced cell death and growth inhibition and up-regulated expression of early growth response protein 1 (EGR-1), whose low expression also carried unfavorable clinical implications.
  • EGR-1 knockdown in MM cells abrogated JUN overexpression-induced MM cell death and growth inhibition, indicating that EGR-1 acts directly downstream of JUN.
  • JUN modulates myeloma cell apoptosis through interacting with EGR-1, which down-regulates Survivin and triggers caspase signaling.

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  • (PMID = 19837979.001).
  • [ISSN] 1528-0020
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / P01 CA055819; United States / NCI NIH HHS / CA / R01 CA113992; United States / NCI NIH HHS / CA / P01 CA55819
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / BIRC5 protein, human; 0 / Boronic Acids; 0 / EGR1 protein, human; 0 / Early Growth Response Protein 1; 0 / Inhibitor of Apoptosis Proteins; 0 / Microtubule-Associated Proteins; 0 / Proto-Oncogene Proteins c-jun; 0 / Pyrazines; 69G8BD63PP / Bortezomib
  • [Other-IDs] NLM/ PMC2803692
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26. Gabrea A, Leif Bergsagel P, Michael Kuehl W: Distinguishing primary and secondary translocations in multiple myeloma. DNA Repair (Amst); 2006 Sep 8;5(9-10):1225-33
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Multiple myeloma (MM) is a malignant post-germinal center tumor of somatically-mutated, isotype-switched plasma cells that accumulate in the bone marrow.
  • It often is preceded by a stable pre-malignant tumor called monoclonal gammopathy of undetermined significance (MGUS), which can sporadically progress to MM.
  • Five recurrent primary translocations involving the immunoglobulin heavy chain (IgH) locus on chromosome 14q32 have been identified in MGUS and MM tumors.
  • A MYC gene is dysregulated by complex translocations and insertions as a very late event during the progression of MM tumors.
  • Since the IgH switch recombination and somatic hypermutation mechanism are turned off in plasma cells and plasma cell tumors, the MYC rearrangements are thought to be mediated by unknown mechanisms that contribute to structural genomic instability in all kinds of tumors.
  • It is hypothesized that secondary translocations not involving a MYC gene can occur at any stage of tumorigenesis, including in pre-malignant MGUS tumor cells.
  • [MeSH-minor] Burkitt Lymphoma / genetics. Chromosome Breakage. Humans. Immunoglobulins / genetics. Lymphoma, B-Cell / genetics. Models, Genetic. Plasmacytoma / genetics


27. Nakamura Y, Sato Y, Ito Y, Maeda T, Takahashi N, Kawai N, Jinnai I, Bessho M, Kinoshita S, Yamamoto T: [POEMS syndrome presenting with transient immunoglobulin isotype switching after successful treatment with autologous peripheral blood stem cell transplantation]. Rinsho Ketsueki; 2007 Aug;48(8):642-6
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  • [Title] [POEMS syndrome presenting with transient immunoglobulin isotype switching after successful treatment with autologous peripheral blood stem cell transplantation].
  • Based on the existence of monoclonal gammopathy of the IgG-lamda type, a slight increase of plasma cells in the bone marrow, and an elevated level of serum vascular endothelial growth factor (VEGF), the diagnosis of POEMS syndrome was made.
  • After peripheral blood stem cell collection by etoposide and G-CSF, the patient received high dose melphalan (200 mg/m2) therapy supported by autologous peripheral blood stem cell transplantation (autoPBSCT).
  • After high-dose chemotherapy with autoPBSCT, the serum VEGF level normalized and the monoclonal IgG-lamda, disappeared.
  • After the autoPBSCT, monoclonal IgG-kappa, protein was detected transiently in serum.
  • The new monoclonal immunoglobulin was considered to be due to normal immune reconstitution after myeloablation rather than alteration of the abnormal plasma cell clone, similarly as oligoclonal immunoglobulins occur in multiple myeloma after autoPBSCT.
  • [MeSH-major] POEMS Syndrome / immunology. POEMS Syndrome / therapy. Peripheral Blood Stem Cell Transplantation

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  • (PMID = 17867301.001).
  • [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
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Immunoglobulin G; 0 / Immunoglobulin kappa-Chains
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28. Argyriou AA: Molecularly targeted therapies for dysimmune neuropathies. Mol Med; 2009 Jul-Aug;15(7-8):283-7
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  • Conventional treatment options, including corticosteroids, intravenous immunoglobulin, or plasma exchange, often fail to treat dysimmune neuropathies, such as chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, and monoclonal gammopathy with its subtypes.
  • Currently, several monoclonal antibodies (MAbs) have been tested in open-label small-sized studies or even in single cases so as to establish future directions in the therapy of diseases of the peripheral nervous system (PNS).
  • Rituximab, an MAb targeting against the B cell surface membrane protein CD20, is the most widely used and promising MAb for the treatment of dysimmune neuropathies, especially for those in which immunoglobulin M (IgM) autoantibodies are pathogenetically involved.
  • [MeSH-major] Antibodies, Monoclonal / therapeutic use. Autoimmune Diseases of the Nervous System / therapy. Drug Delivery Systems / methods. Immunologic Factors / therapeutic use. Polyneuropathies / therapy
  • [MeSH-minor] Antibodies, Monoclonal, Humanized. Antibodies, Monoclonal, Murine-Derived. Antibodies, Neoplasm / therapeutic use. Bevacizumab. Etanercept. Humans. Immunoglobulin G / therapeutic use. Receptors, Tumor Necrosis Factor / therapeutic use. Rituximab

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  • (PMID = 19593413.001).
  • [ISSN] 1528-3658
  • [Journal-full-title] Molecular medicine (Cambridge, Mass.)
  • [ISO-abbreviation] Mol. Med.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / Antibodies, Monoclonal, Murine-Derived; 0 / Antibodies, Neoplasm; 0 / Immunoglobulin G; 0 / Immunologic Factors; 0 / Receptors, Tumor Necrosis Factor; 2S9ZZM9Q9V / Bevacizumab; 3A189DH42V / alemtuzumab; 4F4X42SYQ6 / Rituximab; OP401G7OJC / Etanercept
  • [Number-of-references] 48
  • [Other-IDs] NLM/ PMC2707512
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29. Leone PE, Walker BA, Jenner MW, Chiecchio L, Dagrada G, Protheroe RK, Johnson DC, Dickens NJ, Brito JL, Else M, Gonzalez D, Ross FM, Chen-Kiang S, Davies FE, Morgan GJ: Deletions of CDKN2C in multiple myeloma: biological and clinical implications. Clin Cancer Res; 2008 Oct 01;14(19):6033-41
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  • CDKN2C at 1p32.3 has been identified in myeloma cell lines as the potential target of the deletion.
  • EXPERIMENTAL DESIGN: We analyzed 515 cases of monoclonal gammopathy of undetermined significance (MGUS), smoldering multiple myeloma (SMM), and newly diagnosed multiple myeloma using fluorescence in situ hybridization (FISH) for deletions of CDKN2C.
  • RESULTS: By FISH we identified deletion of 1p32.3 (CDKN2C) in 3 of 66 MGUS (4.5%), 4 of 39 SMM (10.3%), and 55 of 369 multiple myeloma cases (15%).
  • Cases with homozygous deletions of CDKN2C were the most proliferative myelomas, defined by an expression-based proliferation index, consistent with its biological function as a cyclin-dependent kinase inhibitor.
  • [MeSH-minor] Aged. Cell Line, Tumor. Chromosome Mapping / methods. Disease Progression. Heterozygote. Homozygote. Humans. In Situ Hybridization, Fluorescence. Middle Aged. Models, Genetic. Time Factors. Treatment Outcome

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  • (PMID = 18829482.001).
  • [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 Kingdom / Cancer Research UK / / A6308; United Kingdom / Cancer Research UK / /
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / CDKN2C protein, human; 0 / Cyclin-Dependent Kinase Inhibitor p18
  • [Other-IDs] NLM/ PMC2581792; NLM/ UKMS2612
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30. Scudla V, Ordeltova M, Bacovsky J, Vytrasova M, Horak P, Minarik J: The relationship between proliferation and apoptosis in patients with monoclonal gammopathy of undetermined significance or multiple myeloma. Haematologica; 2005 Dec;90(12):1713-4
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  • [Title] The relationship between proliferation and apoptosis in patients with monoclonal gammopathy of undetermined significance or multiple myeloma.
  • Multiple myeloma (MM) is a clonal neoplastic lymphoproliferative disease affecting terminally differentiated B cells i.e. plasma cells characterized by slow proliferation activity and different resistance to apoptosis with latent accumulation of myeloma cells in the bone marrow.
  • We compared plasma cell proliferation and apoptic indices in various phases of MM and in monoclonal gammophaty of untetermined significance.
  • [MeSH-major] Apoptosis. Paraproteinemias / pathology. Plasma Cells / pathology
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cell Division. Clone Cells / pathology. Disease Progression. Female. Humans. Male. Middle Aged. Mitotic Index. Multiple Myeloma / drug therapy. Multiple Myeloma / pathology. Multiple Myeloma / surgery. Peripheral Blood Stem Cell Transplantation. Transplantation, Autologous

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  • (PMID = 16330455.001).
  • [ISSN] 1592-8721
  • [Journal-full-title] Haematologica
  • [ISO-abbreviation] Haematologica
  • [Language] eng
  • [Publication-type] Comparative Study; Letter; Research Support, Non-U.S. Gov't
  • [Publication-country] Italy
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31. Lottersberger F, Panza A, Lucchini G, Longhese MP: Functional and physical interactions between yeast 14-3-3 proteins, acetyltransferases, and deacetylases in response to DNA replication perturbations. Mol Cell Biol; 2007 May;27(9):3266-81
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  • Inactivation of the catalytic subunit of the histone acetyltransferase NuA4 or of its interactor Yng2, besides leading to S-phase defects and persistent checkpoint activation in the presence of genotoxic agents, is lethal for bmh mutants.
  • Conversely, the lack of the histone deacetylase subunit Rpd3 or Sin3 partially suppresses the hypersensitivity to HU of bmh mutants and restores their ability to complete DNA replication in the presence of MMS or HU.
  • These data strongly suggest that reduced acetyltransferase functionality might account for the S-phase defects of bmh mutants in the presence of genotoxic agents.
  • Consistent with a role of 14-3-3 proteins in acetyltransferase and deacetylase regulation, we find that acetylation of H3 and H4 histone tails is reduced in temperature-sensitive bmh mutants shifted to the restrictive temperature.
  • Moreover, Bmh proteins physically interact, directly or indirectly, with the Esa1 acetyltransferase throughout the cell cycle and with the Rpd3 deacetylase specifically during unperturbed S phase and after HU treatment.
  • [MeSH-minor] Cell Cycle Proteins / metabolism. Checkpoint Kinase 2. Enzyme Activation. Genome, Fungal / genetics. Intracellular Signaling Peptides and Proteins. Methyl Methanesulfonate / metabolism. Mutation / genetics. Protein Binding. Protein Subunits / genetics. Protein Subunits / metabolism. Protein-Serine-Threonine Kinases / metabolism. Repressor Proteins / genetics. Repressor Proteins / metabolism. S Phase. Saccharomyces cerevisiae Proteins / genetics. Saccharomyces cerevisiae Proteins / metabolism. Transcription Factors / genetics. Transcription Factors / metabolism

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  • (PMID = 17339336.001).
  • [ISSN] 0270-7306
  • [Journal-full-title] Molecular and cellular biology
  • [ISO-abbreviation] Mol. Cell. Biol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / 14-3-3 Proteins; 0 / Cell Cycle Proteins; 0 / DNA, Fungal; 0 / Intracellular Signaling Peptides and Proteins; 0 / Protein Subunits; 0 / Repressor Proteins; 0 / SIN3 protein, S cerevisiae; 0 / Saccharomyces cerevisiae Proteins; 0 / Transcription Factors; AT5C31J09G / Methyl Methanesulfonate; EC 2.3.1.48 / Esa1 protein, S cerevisiae; EC 2.3.1.48 / Histone Acetyltransferases; EC 2.3.1.48 / NuA4 protein, S cerevisiae; EC 2.7.1.- / RAD53 protein, S cerevisiae; EC 2.7.1.11 / Checkpoint Kinase 2; EC 2.7.11.1 / MEC1 protein, S cerevisiae; EC 2.7.11.1 / Protein-Serine-Threonine Kinases; EC 3.5.1.- / RPD3 protein, S cerevisiae; EC 3.5.1.98 / Histone Deacetylases
  • [Other-IDs] NLM/ PMC1899974
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32. Pratt G: The evolving use of serum free light chain assays in haematology. Br J Haematol; 2008 May;141(4):413-22
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  • The new FLC assay has enabled the detection of monoclonal protein in some patients with non-secretory myeloma and amyloidosis that were previously undetectable.
  • FLC measurements are quantitative, correlating with disease activity, and are an advance in monitoring light chain only multiple myeloma, AL amyloidosis, non-secretory and oligo-secretory multiple myeloma.
  • Serum FLC concentrations also reflect the disease course in the majority of myeloma patients producing intact monoclonal immunoglobulin proteins and have been incorporated into the new response criteria.
  • An abnormal FLC ratio has been shown to be a risk factor for progression of monoclonal gammopathy of undetermined significance, smouldering myeloma and solitary plasmacytoma of bone and is prognostic in multiple myeloma.
  • [MeSH-major] Biomarkers, Tumor / blood. Immunoglobulin Light Chains / blood. Multiple Myeloma / diagnosis
  • [MeSH-minor] Hematologic Neoplasms / diagnosis. Humans. Paraproteinemias / diagnosis. Prognosis

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  • [CommentIn] Br J Haematol. 2008 Oct;143(1):143-5; author reply 145-6 [18671704.001]
  • (PMID = 18318757.001).
  • [ISSN] 1365-2141
  • [Journal-full-title] British journal of haematology
  • [ISO-abbreviation] Br. J. Haematol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Immunoglobulin Light Chains
  • [Number-of-references] 72
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33. Weiss BM, Kuehl WM: Advances in understanding monoclonal gammopathy of undetermined significance as a precursor of multiple myeloma. Expert Rev Hematol; 2010 Apr;3(2):165-74
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  • [Title] Advances in understanding monoclonal gammopathy of undetermined significance as a precursor of multiple myeloma.
  • Monoclonal gammopathy of undetermined significance (MGUS) affects at least 3% of the population above the age of 50 and is the precursor to multiple myeloma (MM), an incurable malignancy of plasma cells.
  • Recent advances in MGUS include: an improved understanding of the pathogenesis of MGUS and its progression to MM, involving molecular events intrinsic to the malignant plasma cell as well as the microenvironment; novel techniques to assess risk for progression to MM using serum-free light-chain analysis and immunophenotyping; and a renewed interest in chemoprevention of MM.
  • In the future, continued improvement in our understanding of MGUS will lead to the development of better biomarkers for prognosis and therapies for chemoprevention of MM.
  • [MeSH-major] Multiple Myeloma / diagnosis. Paraproteinemias / diagnosis

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  • (PMID = 20473362.001).
  • [ISSN] 1747-4094
  • [Journal-full-title] Expert review of hematology
  • [ISO-abbreviation] Expert Rev Hematol
  • [Language] eng
  • [Databank-accession-numbers] ClinicalTrials.gov/ NCT00480363
  • [Grant] United States / Intramural NIH HHS / / Z99 CA999999; United States / Intramural NIH HHS / / ZIA SC006581-26
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Immunoglobulin Light Chains; EC 3.6.5.2 / ras Proteins
  • [Other-IDs] NLM/ NIHMS199922; NLM/ PMC2869099
  • [Keywords] NOTNLM ; MGUS / monoclonal gammopathy of undetermined significance / multiple myeloma / pathogenesis
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34. Condomines M, Hose D, Raynaud P, Hundemer M, De Vos J, Baudard M, Moehler T, Pantesco V, Moos M, Schved JF, Rossi JF, Rème T, Goldschmidt H, Klein B: Cancer/testis genes in multiple myeloma: expression patterns and prognosis value determined by microarray analysis. J Immunol; 2007 Mar 1;178(5):3307-15
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  • We report the expression pattern of CT genes in purified MM cells (MMC) of 64 patients with newly diagnosed MM and12 patients with monoclonal gammopathy of unknown significance, in normal plasma cell and B cell samples, and in 20 MMC lines.
  • [MeSH-major] Antigens, Neoplasm / biosynthesis. Gene Expression Regulation, Neoplastic. Multiple Myeloma / metabolism

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  • (PMID = 17312182.001).
  • [ISSN] 0022-1767
  • [Journal-full-title] Journal of immunology (Baltimore, Md. : 1950)
  • [ISO-abbreviation] J. Immunol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, Neoplasm; 0 / Cancer Vaccines
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35. Mseddi-Hdiji S, Haddouk S, Ben Ayed M, Tahri N, Elloumi M, Baklouti S, Hachicha J, Krichen MS, Bahloul Z, Masmoudi H: [Monoclonal gammapathies in Tunisia: epidemiological, immunochemical and etiological analysis of 288 cases]. Pathol Biol (Paris); 2005 Feb;53(1):19-25
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  • [Title] [Monoclonal gammapathies in Tunisia: epidemiological, immunochemical and etiological analysis of 288 cases].
  • [Transliterated title] Gammapathies monoclonales en Tunisie: analyse épidémiologique, immunochimique et étiologique d'une série de 288 cas.
  • From July 1992 to December 2000, 288 cases of monoclonal gammapathy (MG) were collected at the university hospital of Sfax.
  • The middle age of the patients at the time of the diagnosis was 62 years and 7 months with extremes to 18 months and 99 years and median to 64 years.
  • Among the 270 observations for which aetiology has been established, 73 were classified MG of undetermined significance (MGUS), 160 myeloma (or plasmocytoma) and 37 other malignant MG (Waldenstrom's macroglobulinemia: 13, lymphoma: 9, alpha heavy chains disease: 6, primary amyloidosis: 5, chronic lymphocytic leukaemia: 4).
  • Rheumatological affections (19.2%), infections and renal failure (10% each), haematological and autoimmune diseases (9.6% each) were pathologies most often associated with MGUS.
  • Agarose gel electrophoresis did not show a monoclonal peak in 16% of the cases.
  • IgG is even more predominant in the MGUS group (65.8%).
  • The IgA isotype counts for 20.8% of the cases in our set and the free light chains (kappa or lambda) for 13.6% of the cases whereas the IgM represents 8.7% only of the 288 cases of our set which involves three cases of IgD myeloma and six cases of biclonal gammapathy.

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  • (PMID = 15620605.001).
  • [ISSN] 0369-8114
  • [Journal-full-title] Pathologie-biologie
  • [ISO-abbreviation] Pathol. Biol.
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Immunoglobulin Light Chains; 0 / Immunoglobulin M
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36. Bakker AJ, Mücke M: Gammopathy interference in clinical chemistry assays: mechanisms, detection and prevention. Clin Chem Lab Med; 2007;45(9):1240-3
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  • [Title] Gammopathy interference in clinical chemistry assays: mechanisms, detection and prevention.
  • Monoclonal gammopathy is characterized by the presence of an M-protein in serum or urine that has homogeneous structural and functional properties.
  • Examples of gammopathy interference for the analytes glucose, bilirubin, gamma-glutamyltransferase, urea and ferritin are presented.
  • Modern analyzers can detect unusual changes in absorption during the course of a reaction, and thus the formation of turbidity due to M-proteins.
  • Owing to the unique properties of each M-protein, it is impossible to protect common clinical chemistry test systems completely from gammopathy interference.

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  • [CommentIn] Clin Chem Lab Med. 2009;47(5):614-5 [19278372.001]
  • (PMID = 17635066.001).
  • [ISSN] 1434-6621
  • [Journal-full-title] Clinical chemistry and laboratory medicine
  • [ISO-abbreviation] Clin. Chem. Lab. Med.
  • [Language] ENG
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Immunoglobulin M
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37. Kyle RA, Therneau TM, Rajkumar SV, Larson DR, Plevak MF, Offord JR, Dispenzieri A, Katzmann JA, Melton LJ 3rd: Prevalence of monoclonal gammopathy of undetermined significance. N Engl J Med; 2006 Mar 30;354(13):1362-9
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  • [Title] Prevalence of monoclonal gammopathy of undetermined significance.
  • BACKGROUND: The prevalence of monoclonal gammopathy of undetermined significance (MGUS), a premalignant plasma-cell disorder, among persons 50 years of age or older has not been accurately determined.
  • We used sensitive laboratory techniques to ascertain the prevalence of MGUS in a large population in a well-defined geographic area.
  • Agarose-gel electrophoresis was performed on all serum samples, and any serum sample with a discrete band of monoclonal protein or thought to have a localized band was subjected to immunofixation.
  • MGUS was identified in 694 (3.2 percent) of these persons.
  • Age-adjusted rates were higher in men than in women (4.0 percent vs. 2.7 percent, P<0.001).
  • The prevalence of MGUS was 5.3 percent among persons 70 years of age or older and 7.5 percent among those 85 years of age or older.
  • The concentration of monoclonal immunoglobulin was less than 1.0 g per deciliter in 63.5 percent and at least 2.0 g per deciliter in only 4.5 percent of 694 persons.
  • The concentration of uninvolved immunoglobulins was reduced in 27.7 percent of 447 persons tested, and 21.5 percent of 79 tested had a monoclonal urinary light chain.
  • CONCLUSIONS: Among residents of Olmsted County, Minnesota, MGUS was found in 3.2 percent of persons 50 years of age or older and 5.3 percent of persons 70 years of age or older.

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  • [Copyright] Copyright 2006 Massachusetts Medical Society.
  • [CommentIn] N Engl J Med. 2006 Jun 29;354(26):2832; author reply 2832 [16807426.001]
  • (PMID = 16571879.001).
  • [ISSN] 1533-4406
  • [Journal-full-title] The New England journal of medicine
  • [ISO-abbreviation] N. Engl. J. Med.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA 107476; United States / NCI NIH HHS / CA / CA 62242
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunoglobulin A; 0 / Immunoglobulin G; 0 / Immunoglobulin M
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38. Moreaux J, Veyrune JL, Reme T, De Vos J, Klein B: CD200: a putative therapeutic target in cancer. Biochem Biophys Res Commun; 2008 Feb 1;366(1):117-22
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  • CD200 was recently described as a new prognosis factor in multiple myeloma and acute myeloid leukemia.
  • CD200 is a membrane glycoprotein that imparts an immunoregulatory signal through CD200R, leading to the suppression of T-cell-mediated immune responses.
  • CD200 gene expression in normal or malignant human tissues or cell lines was obtained from the Oncomine Cancer Microarray database, Amazonia database and the ITTACA database.
  • We found significant overexpression of CD200 in renal carcinoma, head and neck carcinoma, testicular cancer, malignant mesothelioma, colon carcinoma, MGUS/smoldering myeloma, and in chronic lymphocytic leukemia compared to their normal cells or their tissue counterparts.
  • [MeSH-minor] Drug Design. Gene Expression Regulation, Neoplastic. Humans. Neoplasm Proteins / metabolism

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  • (PMID = 18060862.001).
  • [ISSN] 1090-2104
  • [Journal-full-title] Biochemical and biophysical research communications
  • [ISO-abbreviation] Biochem. Biophys. Res. Commun.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Biomarkers, Tumor; 0 / Neoplasm Proteins; 0 / antigens, CD200
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39. Chatterjee M, Rancso C, Stühmer T, Eckstein N, Andrulis M, Gerecke C, Lorentz H, Royer HD, Bargou RC: The Y-box binding protein YB-1 is associated with progressive disease and mediates survival and drug resistance in multiple myeloma. Blood; 2008 Apr 1;111(7):3714-22
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  • [Title] The Y-box binding protein YB-1 is associated with progressive disease and mediates survival and drug resistance in multiple myeloma.
  • Current knowledge about molecular mechanisms underlying disease progression and drug resistance in multiple myeloma (MM) is still limited.
  • Immunohistochemical analyses revealed that neither normal bone marrow (BM) plasma cells (PCs), premalignant PCs of patients with monoclonal gammopathy of unknown significance (MGUS), nor MM cells with a mature morphology showed expression of YB-1 in situ.
  • In contrast, YB-1 was strongly expressed in situ in normal PC precursor blasts as well as in a MM subset and in vitro in all of the evaluated MM cell lines.
  • Thus, YB-1 contributes to disease progression, survival, and drug resistance in MM and might therefore provide an attractive therapeutic target.
  • [MeSH-major] Cell Proliferation. DNA-Binding Proteins / biosynthesis. Drug Resistance, Neoplasm. Gene Expression Regulation, Neoplastic. Lymphoid Progenitor Cells / metabolism. Multiple Myeloma / metabolism. Nuclear Proteins / biosynthesis
  • [MeSH-minor] Antibiotics, Antineoplastic / pharmacology. Antibiotics, Antineoplastic / therapeutic use. Apoptosis / drug effects. Apoptosis / genetics. Bone Marrow / metabolism. Bone Marrow / pathology. Cell Line, Tumor. Doxorubicin / pharmacology. Doxorubicin / therapeutic use. Humans. Immunohistochemistry. Ki-67 Antigen / biosynthesis. Ki-67 Antigen / genetics. Plasma Cells / metabolism. Plasma Cells / pathology. RNA, Small Interfering / genetics. Stromal Cells / metabolism. Stromal Cells / pathology. Y-Box-Binding Protein 1

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  • (PMID = 18006704.001).
  • [ISSN] 0006-4971
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 0 / DNA-Binding Proteins; 0 / Ki-67 Antigen; 0 / Nuclear Proteins; 0 / RNA, Small Interfering; 0 / Y-Box-Binding Protein 1; 0 / YBX1 protein, human; 80168379AG / Doxorubicin
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40. Lynch HT, Thomé SD: Familial multiple myeloma. Blood; 2009 Jul 23;114(4):749-50
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  • In this issue of Blood, there are 2 reports on the increased risk of plasma cell disorders in the first-degree relatives of patients with multiple myeloma or MGUS.

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  • [CommentOn] Blood. 2009 Jul 23;114(4):791-5 [19182202.001]
  • [CommentOn] Blood. 2009 Jul 23;114(4):785-90 [19179466.001]
  • (PMID = 19628711.001).
  • [ISSN] 1528-0020
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] eng
  • [Publication-type] Comment; Journal Article
  • [Publication-country] United States
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41. Stewart I, Roddie C, Gill A, Clarkson A, Mirams M, Coyle L, Ward C, Clifton-Bligh P, Robinson BG, Mason RS, Clifton-Bligh RJ: Elevated serum FGF23 concentrations in plasma cell dyscrasias. Bone; 2006 Aug;39(2):369-76
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  • [Title] Elevated serum FGF23 concentrations in plasma cell dyscrasias.
  • Fibroblast growth factor 23 (FGF23) is now recognized as a key regulator of phosphate metabolism.
  • Hypophosphatemic osteomalacia more rarely occurs in non-mesenchymal tumors.
  • We identified elevated serum FGF23 levels in one patient with chronic lymphatic leukemia (CLL) and hypophosphatemia, prompting us to examine FGF23 concentrations in other patients with B-cell neoplasms.
  • FGF23 levels were elevated in several patients with myeloma and monoclonal gammopathy of undetermined significance (MGUS), and were significantly associated with serum paraprotein and beta-2 microglobulin concentrations in these patients.
  • Malignant plasma cells in bone marrow trephines from patients with myeloma showed cytoplasmic expression of FGF23, similar to the cytoplasmic localization of FGF23 already described in mesenchymal tumors associated with oncogenic osteomalacia.
  • We suggest that the relationship between FGF23 and skeletal disease associated with plasma cell dyscrasias deserves further study.
  • [MeSH-major] Fibroblast Growth Factors / blood. Plasma Cells / pathology
  • [MeSH-minor] Aged. Calcium / blood. Fatal Outcome. Female. Humans. Hypophosphatemia / blood. Immunohistochemistry. Leukemia, Lymphocytic, Chronic, B-Cell / diagnosis. Osteomalacia / blood. Phosphates / blood. beta 2-Microglobulin / blood

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  • (PMID = 16644301.001).
  • [ISSN] 8756-3282
  • [Journal-full-title] Bone
  • [ISO-abbreviation] Bone
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Phosphates; 0 / beta 2-Microglobulin; 0 / fibroblast growth factor 23; 62031-54-3 / Fibroblast Growth Factors; SY7Q814VUP / Calcium
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42. Koski AM, Sikiö A, Forslund T: Teriparatide treatment complicated by malignant myeloma. BMJ Case Rep; 2010;2010
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  • We report a case with monoclonal gammopathy of uncertain significance (MGUS) who developed malignant myeloma after teriparatide treatment and we suggest that in addition to malignant myeloma and smouldering myeloma, MGUS should also be considered contraindicated for teriparatide treatment.

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  • [Cites] J Clin Endocrinol Metab. 2004 Jul;89(7):3332-6 [15240611.001]
  • [Cites] Br Med J. 1980 Jun 7;280(6228):1340-4 [6992932.001]
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  • (PMID = 22767690.001).
  • [ISSN] 1757-790X
  • [Journal-full-title] BMJ case reports
  • [ISO-abbreviation] BMJ Case Rep
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 10T9CSU89I / Teriparatide
  • [Other-IDs] NLM/ PMC3027506
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43. Fernández de Larrea C, Rovira M, Mascaró JM Jr, Torras A, Solé M, Lloreta J, Serra N, Cibeira MT, Bladé J: Generalized cutis laxa and fibrillar glomerulopathy resulting from IgG Deposition in IgG-lambda Monoclonal Gammopathy: pulmonary hemorrhage during stem cell mobilization and complete hematological response with bortezomib and dexamethasone therapy. Eur J Haematol; 2009 Feb;82(2):154-8
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  • [Title] Generalized cutis laxa and fibrillar glomerulopathy resulting from IgG Deposition in IgG-lambda Monoclonal Gammopathy: pulmonary hemorrhage during stem cell mobilization and complete hematological response with bortezomib and dexamethasone therapy.
  • The case of a 52-years-old man with generalized acquired cutis laxa associated with IgG-lambda monoclonal gammopathy and nephrotic syndrome with renal failure (due to fibrillar glomerulopathy resulting from IgG deposition) is reported.
  • A peripheral blood autologous stem cell transplant was planned, but the procedure was complicated by severe pulmonary hemorrhage during stem cells mobilization with granulocyte colony-stimulating factor (G-CSF).
  • Finally, the complete hematological response with the disappearance of the toxic M-protein allows the possibility of a long-term benefit with a kidney transplant followed by an autologous bone marrow transplant.

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  • (PMID = 19018863.001).
  • [ISSN] 1600-0609
  • [Journal-full-title] European journal of haematology
  • [ISO-abbreviation] Eur. J. Haematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Boronic Acids; 0 / Immunoglobulin G; 0 / Pyrazines; 143011-72-7 / Granulocyte Colony-Stimulating Factor; 69G8BD63PP / Bortezomib; 7S5I7G3JQL / Dexamethasone
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44. Zheng JM, Chen S, Zhang WQ, Pan XZ, Zhu HX, Jiang WM: [Misdiagnosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome: a case report]. Zhonghua Gan Zang Bing Za Zhi; 2007 Aug;15(8):626-7
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  • [Title] [Misdiagnosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome: a case report].
  • [MeSH-major] Diagnostic Errors. POEMS Syndrome / diagnosis
  • [MeSH-minor] Ascites / complications. Ascites / diagnosis. Humans. Liver Cirrhosis / complications. Liver Cirrhosis / diagnosis. Male. Middle Aged

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  • (PMID = 17711642.001).
  • [ISSN] 1007-3418
  • [Journal-full-title] Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology
  • [ISO-abbreviation] Zhonghua Gan Zang Bing Za Zhi
  • [Language] chi
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] China
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45. Silverman BA, Ku M, Kapur P, Schneider AT: Monoclonal gammopathy in association with allergic disorders of the skin and respiratory tract. Allergy Asthma Proc; 2006 Mar-Apr;27(2):130-9
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  • [Title] Monoclonal gammopathy in association with allergic disorders of the skin and respiratory tract.
  • Monoclonal gammopathy is a condition characterized by the abnormal proliferation of a single clone of plasma cells, which produces a homogeneous monoclonal protein.
  • We report 11 cases of monoclonal gammopathy presenting to practicing allergists (>2.5% of those screened) primarily in association with dermatologic disorders, i.e., urticaria, angioedema, and nonspecific dermatitis, but also with allergic respiratory disorders, i.e., allergic rhinitis, chronic sinusitis, and asthma.
  • Monoclonal gammopathy, angioedema, urticaria, allergic respiratory disorders, and sinusitis could be linked through antigenic stimulation as a trigger, either infectious, as in chronic sinusitis; self-antigens, as in autoimmunity; or the monoclonal gammopathy itself, causing idiotype-anti-idiotype immune complexes and inflammatory disease.
  • The allergist, dermatologist, otolaryngologist, and primary care physician should all maintain a high index of suspicion for the occurrence of monoclonal gammopathy in the "allergic" population.
  • When monoclonal gammopathy is found, the presence of light chains in the urine should be assessed and the patient should be referred for prompt hematology-oncology evaluation with periodic monitoring for the development of plasma cell dyscrasias.
  • Additional prospective study is necessary to determine the true prevalence of monoclonal gammopathy in the population presenting to the practicing allergist.

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  • (PMID = 16724632.001).
  • [ISSN] 1088-5412
  • [Journal-full-title] Allergy and asthma proceedings
  • [ISO-abbreviation] Allergy Asthma Proc
  • [Language] ENG
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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46. van Rhee F: Light-chain MGUS: implications for clinical practice. Lancet; 2010 May 15;375(9727):1670-1
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  • [Title] Light-chain MGUS: implications for clinical practice.
  • [MeSH-major] Immunoglobulin Light Chains / blood. Monoclonal Gammopathy of Undetermined Significance / complications
  • [MeSH-minor] Aged. Aged, 80 and over. Humans. Middle Aged. Multiple Myeloma / etiology. Precancerous Conditions / complications. Precancerous Conditions / diagnosis. Precancerous Conditions / epidemiology. Risk Factors

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  • [CommentOn] Lancet. 2010 May 15;375(9727):1721-8 [20472173.001]
  • (PMID = 20472153.001).
  • [ISSN] 1474-547X
  • [Journal-full-title] Lancet (London, England)
  • [ISO-abbreviation] Lancet
  • [Language] eng
  • [Publication-type] Comment; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Immunoglobulin Light Chains
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47. Maddox JM, Anderson JA, Plews D, Ludlam CA: Management of acquired von Willebrand's syndrome in a patient requiring major surgery. Haemophilia; 2005 Nov;11(6):633-7
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  • We present the case of a patient with acquired von Willebrand's syndrome and a monoclonal gammopathy of undetermined significance who required cystectomy for relapsed transitional cell carcinoma (TCC) of the bladder.
  • We demonstrated that infused von Willebrand factor (VWF) containing factor VIII concentrates had an unacceptably short half-life, but that this was significantly prolonged following combined therapy with plasma exchange and intravenous immunoglobulin (IVIgG).
  • Levels again fell on the fifth postoperative day with the development of a Staphylococcus aureus septicaemia.
  • At this point bleeding occurred from a surgical drain site requiring 'factor VIII inhibitor bypass activity' to secure haemostasis while further plasma exchange and IVIgG were administered.
  • Now 5 years later, there is no evidence of recurrence of the TCC or progression of the monoclonal gammopathy.
  • [MeSH-major] Carcinoma, Transitional Cell / surgery. Urinary Bladder Neoplasms / surgery. von Willebrand Diseases / drug therapy
  • [MeSH-minor] Blood Loss, Surgical / prevention & control. Factor VIII / therapeutic use. Humans. Immunoglobulins, Intravenous / therapeutic use. Male. Middle Aged. Plasma Exchange / methods. Treatment Outcome. von Willebrand Factor / therapeutic use

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  • [CommentIn] Haemophilia. 2006 May;12(3):287-8 [16643216.001]
  • (PMID = 16236115.001).
  • [ISSN] 1351-8216
  • [Journal-full-title] Haemophilia : the official journal of the World Federation of Hemophilia
  • [ISO-abbreviation] Haemophilia
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Immunoglobulins, Intravenous; 0 / von Willebrand Factor; 9001-27-8 / Factor VIII
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48. Krug BC, Schwartz IV, Lopes de Oliveira F, Alegra T, Campos Martins NL, Todeschini LA, Picon PD: The management of Gaucher disease in developing countries: a successful experience in Southern Brazil. Public Health Genomics; 2010;13(1):27-33
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  • [Title] The management of Gaucher disease in developing countries: a successful experience in Southern Brazil.
  • OBJECTIVE: Gaucher disease (GD) is a genetic disease caused by glucocerebrosidase deficiency.
  • GD is treated by enzyme replacement therapy (ERT) with imiglucerase, a high-cost drug provided by the Brazilian Ministry of Health (BMH).
  • This study reports the implementation of the BMH guidelines for GD in the southernmost state of the country.
  • CONCLUSIONS: The model of care of GD patients suggested by the BMH guidelines appears to be cost-effective and could be an example for management of rare diseases in underdeveloped countries.
  • [MeSH-major] Enzyme Replacement Therapy. Gaucher Disease / drug therapy. Glucosylceramidase / therapeutic use
  • [MeSH-minor] Adolescent. Adult. Brazil. Child. Disease Management. Female. Follow-Up Studies. Humans. Liver / drug effects. Liver / pathology. Male. Middle Aged. Patient Compliance. Practice Guidelines as Topic. Spleen / drug effects. Spleen / pathology. Surveys and Questionnaires

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  • [Copyright] Copyright © 2009 S. Karger AG, Basel.
  • (PMID = 19407439.001).
  • [ISSN] 1662-8063
  • [Journal-full-title] Public health genomics
  • [ISO-abbreviation] Public Health Genomics
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Switzerland
  • [Chemical-registry-number] EC 3.2.1.45 / Glucosylceramidase; EC 3.2.1.45 / imiglucerase
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49. Manganelli R, Iannaccone S, Ferbo U, De Simone W: [Diagnostic pathway of an unusual case of nephrotic syndrome: immunotactoid glomerulopathy]. G Ital Nefrol; 2010 Nov-Dec;27(6):668-73
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  • It is clinically characterized by the presence of glomerular proteinuria in the nephrotic range, microscopic hematuria and hypertension, and is often associated with hypocomplementemia, monoclonal gammopathy, and lymphoprolipherative disorders.
  • Immunofluorescence showed IgG deposits with monoclonal kappa light chain restriction as well as C3 and C1q deposits.
  • The final diagnosis was nephrotic syndrome caused by immunotactoid glomerulopathy.
  • The clinical diagnosis of immunotactoid glomerulopathy is based on pathological, clinical and hematological features and requires the exclusion of other diseases that are associated with organized glomerular deposits.
  • [MeSH-major] Glomerulonephritis, Membranoproliferative / diagnosis. Nephrotic Syndrome / diagnosis
  • [MeSH-minor] Complement C3 / deficiency. Diagnosis, Differential. Disease Progression. Female. Glucocorticoids / therapeutic use. Humans. Immunologic Factors / deficiency. Middle Aged. Prednisone / therapeutic use. Proteinuria / etiology. Treatment Outcome

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  • (PMID = 21132650.001).
  • [ISSN] 0393-5590
  • [Journal-full-title] Giornale italiano di nefrologia : organo ufficiale della Società italiana di nefrologia
  • [ISO-abbreviation] G Ital Nefrol
  • [Language] ita
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Complement C3; 0 / Glucocorticoids; 0 / Immunologic Factors; VB0R961HZT / Prednisone
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50. Manousaridis I, Loeser C, Goerdt S, Hassel JC: Managing scleromyxedema with intravenous immunoglobulin: acute worsening of scleromyxedema with biclonal gammopathy. Acta Dermatovenerol Alp Pannonica Adriat; 2010 Dec;19(4):15-9
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  • [Title] Managing scleromyxedema with intravenous immunoglobulin: acute worsening of scleromyxedema with biclonal gammopathy.
  • Scleromyxedema is a rare chronic cutaneous mucinosis usually associated with a monoclonal gammopathy and underlying systemic disease.
  • The etiology of the disease is not known.
  • We report a case of refractory scleromyxedema in a 63-year-old man with a biclonal IgG and IgM λ-gammopathy.

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  • (PMID = 21390475.001).
  • [ISSN] 1581-2979
  • [Journal-full-title] Acta dermatovenerologica Alpina, Pannonica, et Adriatica
  • [ISO-abbreviation] Acta Dermatovenerol Alp Pannonica Adriat
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Slovenia
  • [Chemical-registry-number] 0 / Immunoglobulin G; 0 / Immunoglobulin M; 0 / Immunoglobulins, Intravenous; 0 / Immunologic Factors
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51. Edwards BJ, Langman CB, Bunta AD, Vicuna M, Favus M: Secondary contributors to bone loss in osteoporosis related hip fractures. Osteoporos Int; 2008 Jul;19(7):991-9
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  • Secondary causes for bone loss are present in more than 80% of patients with hip fractures, and therefore, assessment of Vitamin D status, disorders in calcium absorption and excretion, monoclonal gammopathies, and renal function should be performed.
  • Assessment for vitamin D, renal and parathyroid status, calcium absorption, and plasma cell disorders.
  • More than 80% of HFx had at least one previously undiagnosed condition, with vitamin D insufficiency (61%), chronic kidney disease (16%) (CKD), monoclonal gammopathy (6%), and low calcium absorption (5%) being the most common.
  • [MeSH-minor] Aged. Aged, 80 and over. Female. Femur Neck / diagnostic imaging. Hip / diagnostic imaging. Humans. Hyperthyroidism / epidemiology. Kidney Diseases / epidemiology. Lumbar Vertebrae / diagnostic imaging. Male. Neoplasms, Plasma Cell / epidemiology. Prevalence. Radiography. Vitamin D Deficiency / epidemiology

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  • (PMID = 18180974.001).
  • [ISSN] 0937-941X
  • [Journal-full-title] Osteoporosis international : a journal established as result of cooperation between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA
  • [ISO-abbreviation] Osteoporos Int
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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52. Chim CS, Kwong YL, Liang R: Gene hypermethylation in multiple myeloma: lessons from a cancer pathway approach. Clin Lymphoma Myeloma; 2008 Dec;8(6):331-9
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  • Multiple myeloma (MM) is an incurable plasma cell neoplasm.
  • Pathogenesis involves upregulation of D-type cyclins and activation of oncogenes, but little is known about the role of tumor suppressor genes.
  • Based on the cancer pathway approach, the following data on the involvement of cell cycle control, intrinsic tumor suppressor, and cell signaling were derived.
  • Lastly, apart from being implicated in the progression from monoclonal gammopathy of unknown significance to MM, aberrant gene promoter methylation might also account for late disease progression in MM.
  • [MeSH-minor] Cell Cycle. DNA, Neoplasm / metabolism. Disease Progression. Genes, Tumor Suppressor. Humans. Models, Biological. Promoter Regions, Genetic. Signal Transduction


53. Martín MG, Romero Colás MS, Dourdil Sahún MV, Olave P, Alba PR, Banzo JB: BaselinTc99-MIBI scanning predicts survival in multiple myeloma and helps to differentiate this disease from monoclonal gammopathy of unknown significance. Haematologica; 2005 Aug;90(8):1141-3
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  • [Title] BaselinTc99-MIBI scanning predicts survival in multiple myeloma and helps to differentiate this disease from monoclonal gammopathy of unknown significance.
  • We performed baselineTc(99)-MIBI scanning in 43 patients with multiple myeloma (MM) and in 31 with monoclonal gammopathy of unknown significance (MGUS) patients.
  • MGUS patients had normal or very low scores.
  • [MeSH-minor] Diagnosis, Differential. Humans. Predictive Value of Tests. Radiopharmaceuticals. Survival Analysis

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  • (PMID = 16079119.001).
  • [ISSN] 1592-8721
  • [Journal-full-title] Haematologica
  • [ISO-abbreviation] Haematologica
  • [Language] eng
  • [Publication-type] Letter
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 971Z4W1S09 / Technetium Tc 99m Sestamibi
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54. Kurzen C, Kunz I, Nigg C: [Blue discoloration of hands, numbness of feet, indolent cervical lymph node swelling in a 73-year-old man]. Praxis (Bern 1994); 2006 Oct 11;95(41):1589-93
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  • A 73-years old patient came to our outpatient clinic because of a blue discoloration of his hands.
  • In 1996 and 2001 cervical lymph node resections were done because of localized angiofollicular lymphnode hyperplasia (Castleman's disease).
  • The laboratory values confirmed a systemic inflammatory reaction, a hypothyreosis and a monoclonal gammopathy.
  • So all the criterias for a POEMS syndrome (special form of multiple myeloma) were met with polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes.
  • The generalized lymphadenopathy corresponded histologically to the prior mentioned Castleman's disease.
  • The patient responded well to systemic glucocorticoid treatment and today he is asymptomatic.
  • [MeSH-major] Foot / innervation. Giant Lymph Node Hyperplasia / diagnosis. Hand Dermatoses / etiology. Hyperpigmentation / etiology. Hypesthesia / etiology
  • [MeSH-minor] Aged. Diagnosis, Differential. Drug Administration Schedule. Humans. Male. Neck. Prednisone / administration & dosage. Recurrence

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  • (PMID = 17080761.001).
  • [ISSN] 1661-8157
  • [Journal-full-title] Praxis
  • [ISO-abbreviation] Praxis (Bern 1994)
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] VB0R961HZT / Prednisone
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55. Marini A, Fenk R, Plettenberg H, Ruzicka T, Haas R, Hengge UR: [Rare types of vasculitis as markers of plasmocytoma]. Hautarzt; 2006 Feb;57(2):137-43
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  • The results of all examinations and laboratory tests considered together suggested a diagnosis of necrotizing leukocytoclastic vasculitis.
  • Common etiological factors include bacterial, viral or drug antigens, chronic infections (hepatitis B and C), non-Hodgkin lymphomas (monoclonal gammopathy, multiple myeloma), leukemia (hairy cell leukemia), and tumors (bronchial, breast, and gastric cancer) and also connective tissue disorders.
  • [MeSH-major] Plasmacytoma / complications. Plasmacytoma / diagnosis. Skin Neoplasms / complications. Skin Neoplasms / diagnosis. Vasculitis / diagnosis. Vasculitis / etiology
  • [MeSH-minor] Aged. Diagnosis, Differential. Female. Humans. Male. Middle Aged. Rare Diseases / diagnosis. Rare Diseases / etiology

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  • (PMID = 15657729.001).
  • [ISSN] 0017-8470
  • [Journal-full-title] Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete
  • [ISO-abbreviation] Hautarzt
  • [Language] ger
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56. Ross FM, Chiecchio L, Dagrada G, Protheroe RK, Stockley DM, Harrison CJ, Cross NC, Szubert AJ, Drayson MT, Morgan GJ, UK Myeloma Forum: The t(14;20) is a poor prognostic factor in myeloma but is associated with long-term stable disease in monoclonal gammopathies of undetermined significance. Haematologica; 2010 Jul;95(7):1221-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The t(14;20) is a poor prognostic factor in myeloma but is associated with long-term stable disease in monoclonal gammopathies of undetermined significance.
  • A large series of plasma cell dyscrasias (n=2207) was examined for translocations which deregulate the MAF genes, t(14;20)(q32;q12) and t(14;16)(q32;q23), and their disease behavior was compared to a group characterized by the t(4;14)(p16;q32) where CCND2 is also up-regulated.
  • The t(14;20) showed low prevalence in myeloma (27/1830, 1.5%) and smoldering myeloma (1/148, <1%) with a higher incidence in MGUS (9/193, 5% P=0.005).
  • Strong associations with del(13) (76%), non-hyperdiploidy (83%) and gain of 1q (58%) were seen but no association with an IgA M-protein or absence of bone disease was noted.
  • All three translocations were associated with poor outcome in myeloma, but strikingly all t(14;20) MGUS/smoldering myeloma cases (n=10) had stable, low level disease.
  • In contrast, the 10 t(14;16) and 25 t(4;14) MGUS/smoldering myeloma cases were associated with both evolving and non-evolving disease.
  • None of the associated genetic abnormalities helped to predict for progression from MGUS or smoldering myeloma.
  • [MeSH-major] Monoclonal Gammopathy of Undetermined Significance / genetics. Multiple Myeloma / genetics. Translocation, Genetic
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Disease Progression. Female. Humans. Male. Middle Aged. Prognosis

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  • (PMID = 20410185.001).
  • [ISSN] 1592-8721
  • [Journal-full-title] Haematologica
  • [ISO-abbreviation] Haematologica
  • [Language] eng
  • [Grant] United Kingdom / Medical Research Council / / G0100132
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Italy
  • [Other-IDs] NLM/ PMC2895050
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57. Asherson RA, Davidge-Pitts MC, Wypkema E: "Primary" antiphospholipid syndrome evolving into Waldenstrom's macroglobulinaemia: a case report. Clin Rheumatol; 2007 Feb;26(2):278-80
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  • This case illustrates the importance of this investigation in any middle-aged patient presenting with an antiphospholipid syndrome and a monoclonal gammopathy This finding might presage the development of a more serious condition, even years later (as in our patient).

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  • (PMID = 16547696.001).
  • [ISSN] 0770-3198
  • [Journal-full-title] Clinical rheumatology
  • [ISO-abbreviation] Clin. Rheumatol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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58. Kaufmann H, Ackermann J, Odelga V, Sagaster V, Nösslinger T, Pfeilstöcker M, Keck A, Ludwig H, Gisslinger H, Drach J: Cytogenetic patterns in multiple myeloma after a phase of preceding MGUS. Eur J Clin Invest; 2008 Jan;38(1):53-60
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Cytogenetic patterns in multiple myeloma after a phase of preceding MGUS.
  • BACKGROUND: Presenting the same histological diagnosis, multiple myeloma (MM) shows a large genomic variety, resulting in variable times of overall survival.
  • MATERIALS AND METHODS: To investigate major cytogenetic categories (any 14q-translocation, t(11;14), t(4;14), 13q-deletions, 17p-deletions) and their clinical consequences in MM after a pre-existing monoclonal gammopathy (MM post-MGUS), we performed a comparative analysis of 41 patients with MM post-MGUS and 287 patients with unknown prior history MM (U-MM).
  • RESULTS: In MM post-MGUS, a t(11;14) was found to be more frequent than in U-MM (24% vs. 14%) and it was associated with significantly shortened survival (24 months vs. 70 months in U-MM; P = 0.01).
  • MM post-MGUS was further characterized by a higher frequency of 13q-deletions only (absence of all other specific abnormalities; 28% vs. 12% in U-MM; P = 0.02).
  • A 13q-deletion only was an indicator of long survival in MM post-MGUS (median not yet reached) as opposed to U-MM (median survival, 29 months; P = 0.001).
  • 17p-deletions were infrequent in MM post-MGUS (3% vs. 16% in U-MM; P = 0.04).
  • CONCLUSIONS: Our data suggest that t(11;14) and 13q-deletions have distinct prognostic implications in the context of MM post-MGUS.
  • [MeSH-major] Chromosome Deletion. Monoclonal Gammopathy of Undetermined Significance / complications. Multiple Myeloma / genetics. Translocation, Genetic

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  • (PMID = 18173551.001).
  • [ISSN] 1365-2362
  • [Journal-full-title] European journal of clinical investigation
  • [ISO-abbreviation] Eur. J. Clin. Invest.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
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59. Pineda-Roman M, Bolejack V, Arzoumanian V, Anaissie E, van Rhee F, Zangari M, Walker R, Hollmig K, Shaughnessy JD Jr, Epstein J, Krishna S, Crowley J, Barlogie B: Complete response in myeloma extends survival without, but not with history of prior monoclonal gammopathy of undetermined significance or smouldering disease. Br J Haematol; 2007 Feb;136(3):393-9
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  • [Title] Complete response in myeloma extends survival without, but not with history of prior monoclonal gammopathy of undetermined significance or smouldering disease.
  • The tandem transplant trial, Total Therapy 2, enrolled 668 patients, who were randomised up-front to thalidomide (THAL) or no THAL; 56 patients were identified as having had, for at least 6 months prior to initiation of therapy, monoclonal gammopathy of undetermined significance (MGUS, n = 21), smouldering MM (SMM, n = 22) or solitary plasmacytoma of bone (SPC, n = 13).
  • The clinical characteristics and outcomes of patients with such 'evolved' MM (E-MM) and of those with 'unknown' prior history (U-MM) were compared.
  • Fewer patients with MGUS/SMM-E-MM had anaemia or renal failure; CR was lower (22% vs. 48%) but 4-year estimates of event-free survival (54% vs. 56% with U-MM) and overall survival (65% vs. 70% with U-MM) were similar to those with SPC-E-MM or U-MM.
  • In comparison with U-MM, E-MM evolved from MGUS/SMM was associated with lower CR rate without adversely affecting survival.
  • [MeSH-major] Antimetabolites / therapeutic use. Bone Neoplasms / therapy. Monoclonal Gammopathy of Undetermined Significance / therapy. Multiple Myeloma / therapy. Plasmacytoma / therapy. Thalidomide / therapeutic use
  • [MeSH-minor] Aged. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Male. Peripheral Blood Stem Cell Transplantation. Proportional Hazards Models. Remission Induction. Survival Analysis. Transplantation, Autologous

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  • (PMID = 17156398.001).
  • [ISSN] 0007-1048
  • [Journal-full-title] British journal of haematology
  • [ISO-abbreviation] Br. J. Haematol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA55819
  • [Publication-type] Journal Article; Randomized Controlled Trial; Research Support, N.I.H., Extramural
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antimetabolites; 4Z8R6ORS6L / Thalidomide
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60. Mahapatra M, Mishra P, Makharia G, Kumar R, Saxena R: Acquired von Willebrand's disease associated with gastrointestinal angiodysplasia and monoclonal gammopathy. J Assoc Physicians India; 2006 Dec;54:963
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  • [Title] Acquired von Willebrand's disease associated with gastrointestinal angiodysplasia and monoclonal gammopathy.

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  • (PMID = 17334020.001).
  • [ISSN] 0004-5772
  • [Journal-full-title] The Journal of the Association of Physicians of India
  • [ISO-abbreviation] J Assoc Physicians India
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] India
  • [Chemical-registry-number] 0 / Immunoglobulins, Intravenous
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61. Kyle RA, Rajkumar SV: Monoclonal gammopathy of undetermined significance. Clin Lymphoma Myeloma; 2005 Sep;6(2):102-14
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  • [Title] Monoclonal gammopathy of undetermined significance.
  • Monoclonal gammopathy of undetermined significance (MGUS) is characterized by the presence of a monoclonal protein (M-protein) without evidence of multiple myeloma (MM), Waldenstrom's macroglobulinemia (WM), amyloidosis (AL), or a related plasma cell proliferative disorder.
  • Monoclonal gammopathy of undetermined significance is found in approximately 3% of persons > 70 years of age and in about 1% of those > 50 years old.
  • In a series of 1384 patients from Southeastern Minnesota in whom MGUS was diagnosed at Mayo Clinic from 1960 through 1994, the risk of progression was 1% per year.
  • This risk of progression continued even after > or = 25 years of a stable M-protein.
  • The presence of a urine M-protein or the reduction of > or = 1 uninvolved immunoglobulins was not a risk factor for disease progression.
  • Patients must be monitored for progressive disease throughout their lives.
  • Variants of MGUS consist of IgM MGUS, biclonal gammopathies, triclonal gammopathies, idiopathic Bence Jones (light-chain) proteinuria, and IgD MGUS.
  • Monoclonal gammopathy of undetermined significance may be associated with many disorders, including lymphoproliferative diseases, leukemia, von Willebrand's disease, connective tissue diseases, and neurologic disorders.
  • [MeSH-minor] Aged. Amyloidosis / blood. Amyloidosis / classification. Amyloidosis / epidemiology. Disease Progression. Female. Humans. Immunoglobulin A / blood. Immunoglobulin Light Chains / blood. Immunoglobulin M / blood. Male. Middle Aged. Retrospective Studies. Risk Factors

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  • (PMID = 16231848.001).
  • [ISSN] 1557-9190
  • [Journal-full-title] Clinical lymphoma & myeloma
  • [ISO-abbreviation] Clin Lymphoma Myeloma
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA 107476; United States / NCI NIH HHS / CA / CA 62242
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, P.H.S.; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunoglobulin A; 0 / Immunoglobulin Light Chains; 0 / Immunoglobulin M
  • [Number-of-references] 103
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62. Gregersen H, Sørensen HT, Engebjerg MC, Jensen P, Severinsen MT, Nørgaard M: Survival of cancer patients with prior monoclonal gammopathy of undetermined significance. Eur J Intern Med; 2010 Dec;21(6):564-8
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  • [Title] Survival of cancer patients with prior monoclonal gammopathy of undetermined significance.
  • INTRODUCTION: It is unknown whether a prior diagnosis of monoclonal gammopathy of undetermined significance (MGUS) affects cancer survival.
  • DESIGN AND METHODS: We linked data on 1652 cases of MGUS diagnosed during 1978-2006 in North Jutland County, Denmark with the Danish Cancer Registry and the National Patient Registry to obtain information on survival of cancer patients with a previous MGUS compared with that of cancer patients without MGUS, matched on cancer type, age, sex and year of diagnosis.
  • RESULTS: We included 323 cancer patients with previously detected MGUS and 3154 comparison cancer patients without MGUS.
  • The 5-year survival probability was 26.2% (95% CI, 21.2%-31.5%) in cancer patients with MGUS and 30.5% (28.8%-32.1%) in cancer patients without MGUS.
  • Survival following a diagnosis of multiple myeloma, the cancer site of main interest, did not differ according to a preceding MGUS diagnosis.
  • Among patients with immune-related cancers (liver, cervix, malignant melanoma and non-melanoma skin cancers), a preceding MGUS diagnosis was associated with reduced survival (adjusted MRR=1.49 (95% CI: 0.96-2.31)).
  • In contrast, for other non-haematological cancers a prior MGUS diagnosis was associated with a lower MRR (0.78 (95% CI, 0.63-0.96)).
  • CONCLUSION: Our study does not indicate that previously detected MGUS is a prognostic factor for cancer survival in general.
  • [MeSH-major] Monoclonal Gammopathy of Undetermined Significance / mortality. Multiple Myeloma / mortality. Neoplasms / mortality

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  • [Copyright] Copyright © 2010 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
  • (PMID = 21111946.001).
  • [ISSN] 1879-0828
  • [Journal-full-title] European journal of internal medicine
  • [ISO-abbreviation] Eur. J. Intern. Med.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Netherlands
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63. Golombick T, Diamond T: The potential role of curcumin (diferuloylmethane) in plasma cell dyscrasias/paraproteinemia. Biologics; 2008 Mar;2(1):161-3
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  • [Title] The potential role of curcumin (diferuloylmethane) in plasma cell dyscrasias/paraproteinemia.
  • Plasma cell dyscrasias, most commonly associated with paraproteinemia, are a diverse group of diseases.
  • Monoclonal gammopathy of undefined significance (MGUS) can precede multiple myeloma, a progressive neoplastic disease.
  • MGUS occurs in association with a variety of other diseases and currently no treatment is recommended but rather "watchful waiting".
  • Given that the size of the M-protein is a risk factor for disease progression, early intervention with the aim of reducing the paraprotein load would provide an innovative therapeutic tool.
  • As a natural product, this has exciting potential in the treatment of plasma cell dyscrasias.

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  • [Cites] Br J Haematol. 2006 Sep;134(6):573-89 [16938117.001]
  • [Cites] Clin Cancer Res. 2004 Oct 15;10(20):6847-54 [15501961.001]
  • (PMID = 19707439.001).
  • [ISSN] 1177-5475
  • [Journal-full-title] Biologics : targets & therapy
  • [ISO-abbreviation] Biologics
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] New Zealand
  • [Other-IDs] NLM/ PMC2727780
  • [Keywords] NOTNLM ; MGUS / curcumin / myeloma / paraproteinemia / plasma cell dyscrasias
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64. Kumar S, Rajkumar SV, Kimlinger T, Greipp PR, Witzig TE: CD45 expression by bone marrow plasma cells in multiple myeloma: clinical and biological correlations. Leukemia; 2005 Aug;19(8):1466-70
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  • [Title] CD45 expression by bone marrow plasma cells in multiple myeloma: clinical and biological correlations.
  • Multiple myeloma (MM) is characterized by accumulation of clonal plasma cells (PCs).
  • We studied CD45 expression on MM PCs by flow cytometry, correlating it to important biological disease characteristics.
  • A total of 75 patients with untreated MM (29), relapsed MM (17), smoldering MM (12), and monoclonal gammopathy of undetermined significance (MGUS) (17) were studied.
  • The proportion of PCs expressing CD45 was higher among those with early disease (MGUS or smoldering MM) compared to those with advanced disease (new or relapsed MM) (43 vs 22%; P=0.005).
  • Among those with advanced disease, patients with bone lesions had a lower percentage of CD45-positive (CD45+) PCs; 14 vs 34% (P=0.02).
  • [MeSH-major] Antigens, CD45 / analysis. Multiple Myeloma / pathology. Plasma Cells / chemistry
  • [MeSH-minor] Antigens, CD / analysis. Antigens, Neoplasm / analysis. Bone Marrow Cells. Bone Neoplasms / chemistry. Cell Adhesion Molecules / analysis. Cell Count. Disease Progression. Flow Cytometry. Humans. Neovascularization, Pathologic. Survival Analysis

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  • [Copyright] Leukemia (2005) 19, 1466-1470.
  • (PMID = 15959533.001).
  • [ISSN] 0887-6924
  • [Journal-full-title] Leukemia
  • [ISO-abbreviation] Leukemia
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA100080; United States / NCI NIH HHS / CA / CA107476; United States / NCI NIH HHS / CA / CA62242; United States / NCI NIH HHS / CA / CA93842; United States / NCI NIH HHS / CA / CA97274; United States / NCI NIH HHS / CN / CN65125
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Antigens, Neoplasm; 0 / Cell Adhesion Molecules; EC 3.1.3.48 / Antigens, CD45
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65. Sevketoglu E, Hatipoglu S, Ayan I, Dogan O, Salihoglu B: Case report: POEMS syndrome in childhood. J Pediatr Hematol Oncol; 2008 Mar;30(3):235-8
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  • POEMS syndrome is a rare multisystem disorder, which is characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes.
  • We report a 15-year-old girl with POEMS syndrome, who developed growth retardation, delayed puberty, gradually increasing abdominal distention, brown skin pigmentation, hypogonadism, hepatosplenomegaly, lympadenomegaly, monoclonal gammopathy, and anemia.
  • [MeSH-major] Hematopoietic Stem Cell Transplantation. POEMS Syndrome / diagnosis. POEMS Syndrome / therapy. Steroids / therapeutic use
  • [MeSH-minor] Abdomen / pathology. Adolescent. Anemia / diagnosis. Diagnosis, Differential. Female. Growth Disorders / diagnosis. Hepatomegaly / diagnosis. Humans. Hypogonadism / diagnosis. Paraproteinemias / diagnosis. Pigmentation Disorders / diagnosis. Pigmentation Disorders / therapy. Predictive Value of Tests. Puberty, Delayed / diagnosis. Treatment Outcome

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  • (PMID = 18376288.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Steroids
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66. Kyle RA, Rajkumar SV: Monoclonal gammopathies of undetermined significance. Best Pract Res Clin Haematol; 2005;18(4):689-707
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  • [Title] Monoclonal gammopathies of undetermined significance.
  • The monoclonal gammopathies include multiple myeloma (MM), monoclonal gammopathy of undetermined significance (MGUS), primary systemic amyloidosis (AL), and Waldenström's macroglobulinemia (WM).
  • At Mayo Clinic, almost 60% of patients with a monoclonal gammopathy have MGUS.
  • MGUS is characterized by the presence of a serum monoclonal protein value <3 g/dL, fewer than 10% plasma cells in the bone marrow, no or a small amount of monoclonal protein in the urine, and absence of lytic bone lesions, anemia, hypercalcemia, or renal insufficiency related to the plasma-cell proliferative process.
  • During long-term follow-up of 241 patients with MGUS seen at Mayo Clinic from 1956 to 1970, MM, WM, AL, or a related disorder developed in 64.
  • To confirm the findings, we conducted a population-based study on MGUS in the 11 counties of southeastern Minnesota from 1960 to 1994.
  • The risk of progression to a malignant plasma-cell disorder was 1% per year.
  • [MeSH-major] Monoclonal Gammopathy of Undetermined Significance
  • [MeSH-minor] Cell Transformation, Neoplastic. Diagnosis, Differential. Disease Progression. Humans

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  • (PMID = 16026745.001).
  • [ISSN] 1521-6926
  • [Journal-full-title] Best practice & research. Clinical haematology
  • [ISO-abbreviation] Best Pract Res Clin Haematol
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA 62242
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, P.H.S.; Review
  • [Publication-country] England
  • [Number-of-references] 80
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67. Centers for Disease Control and Prevention (CDC): Suicide trends and characteristics among persons in the Guaraní Kaiowá and Nandeva communities--Mato Grosso do Sul, Brazil, 2000-2005. MMWR Morb Mortal Wkly Rep; 2007 Jan 12;56(1):7-9
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  • During 1975-2000, the infant mortality rate decreased, and overall life expectancy increased in Mato Grosso do Sul; however, suicide increased as a proportion of overall mortality among the Kaiowá and Nandeva communities of the Guaraní population.
  • In 2000, the National Health Foundation (FUNASA) of the Brazilian Ministry of Health (BMH) initiated a study of suicide trends and characteristics in these two Guaraní communities; data were collected during 2000-2005, and epidemiologic assistance was provided by CDC.
  • To decrease suicide rates, BMH initiated research and prevention programs among the Guaraní, and the Guaraní initiated measures to increase their economic self-sufficiency.

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  • (PMID = 17218936.001).
  • [ISSN] 1545-861X
  • [Journal-full-title] MMWR. Morbidity and mortality weekly report
  • [ISO-abbreviation] MMWR Morb. Mortal. Wkly. Rep.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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68. Hutchison CA, Basnayake K, Cockwell P: Serum free light chain assessment in monoclonal gammopathy and kidney disease. Nat Rev Nephrol; 2009 Nov;5(11):621-8
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  • [Title] Serum free light chain assessment in monoclonal gammopathy and kidney disease.
  • Abnormalities of immunoglobulin free light chains (FLCs) are frequently present in patients with monoclonal gammopathies and can cause kidney disease.
  • The recent introduction of highly sensitive immunoassays that measure FLCs to levels below those present in normal individuals has provided a new tool for diagnosis and management in this setting.
  • Here, we review the biology of FLC production in health and disease, and the utility of FLC immunoassays in the assessment of monoclonal gammopathies in kidney disease.

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  • (PMID = 19786994.001).
  • [ISSN] 1759-507X
  • [Journal-full-title] Nature reviews. Nephrology
  • [ISO-abbreviation] Nat Rev Nephrol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Immunoglobulin Light Chains
  • [Number-of-references] 60
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69. Kristinsson SY, Goldin LR, Björkholm M, Turesson I, Landgren O: Risk of solid tumors and myeloid hematological malignancies among first-degree relatives of patients with monoclonal gammopathy of undetermined significance. Haematologica; 2009 Aug;94(8):1179-81
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  • [Title] Risk of solid tumors and myeloid hematological malignancies among first-degree relatives of patients with monoclonal gammopathy of undetermined significance.

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  • (PMID = 19546435.001).
  • [ISSN] 1592-8721
  • [Journal-full-title] Haematologica
  • [ISO-abbreviation] Haematologica
  • [Language] ENG
  • [Grant] United States / Intramural NIH HHS / /
  • [Publication-type] Letter; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't
  • [Publication-country] Italy
  • [Other-IDs] NLM/ PMC2719044
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70. Shimojima Y, Matsuda M, Gono T, Ishii W, Fushimi T, Hoshii Y, Yamada T, Ikeda S: Correlation between serum levels of free light chain and phenotype of plasma cells in bone marrow in primary AL amyloidosis. Amyloid; 2005 Mar;12(1):33-40
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  • [Title] Correlation between serum levels of free light chain and phenotype of plasma cells in bone marrow in primary AL amyloidosis.
  • To investigate whether there is a correlation between subtypes of plasma cells in the bone marrow and the production of M-protein, flow cytometry and serum free light chain (FLC) analyses were carried out in 17 patients with primary systemic AL amyloidosis (mean age, 59.9+/-8.8 years) and controls with M-protein (MGUS controls, n=6) and without it (negative controls, n=9).
  • The patients showed a significantly higher value in the serum predominant FLC:serum creatinine ratio (43.8+/-63.2) and CD38++ CD19- CD56+ subpopulation (monoclonal plasma cells) (2.57+/-5.35%) than either the negative (p<0.0005 and p<0.001, respectively) or MGUS controls (p<0.05).
  • With respect to maturation of plasma cells in the bone marrow, the intermediate (MPC-1+ CD45- CD49e-) and mature (MPC-1+ CD45+ CD49e-) subtypes were significantly higher (49.2+/-23.2%, p<0.005) and lower (27.6+/-21.3%, p<0.005) in the patients than in the negative controls, respectively.
  • The serum predominant FLC:serum creatinine ratio was elevated in parallel with an increase in CD38++ CD19- CD56+ and MPC-1+ CD45- CD49e- cells and a decrease in mature subtypes (MPC-1+ CD45+ CD49e- and MPC-1+ CD45+ CD49e+ cells), There was a significantly positive correlation between the serum predominant FLC:serum creatinine ratio and either CD38++ CD19- CD56+ (r=0.510, p<0.05) or MPC-1+ CD45- CD49e- cells (r=0.481, p<0.05).
  • In primary AL amyloidosis M-protein is probably produced by increased monoclonal plasma cells in the bone marrow, particularly by the intermediate subpopulation with a phenotype of MPC-1+ CD45- CD49e-.
  • [MeSH-major] Amyloidosis / blood. Antigens, CD / analysis. Bone Marrow Cells / pathology. Immunoglobulin Light Chains / blood. Plasma Cells / pathology

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  • (PMID = 16076609.001).
  • [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] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Immunoglobulin Light Chains; 0 / Myeloma Proteins; 0 / multiple myeloma M-proteins
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71. Landgren O, Katzmann JA, Hsing AW, Pfeiffer RM, Kyle RA, Yeboah ED, Biritwum RB, Tettey Y, Adjei AA, Larson DR, Dispenzieri A, Melton LJ 3rd, Goldin LR, McMaster ML, Caporaso NE, Rajkumar SV: Prevalence of monoclonal gammopathy of undetermined significance among men in Ghana. Mayo Clin Proc; 2007 Dec;82(12):1468-73
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  • [Title] Prevalence of monoclonal gammopathy of undetermined significance among men in Ghana.
  • OBJECTIVE: To determine the prevalence of monoclonal gammopathy of undetermined significance (MGUS), a precursor of multiple myeloma (MM), in Ghanaian men vs white men and to test for evidence to support an underlying race-related predisposition of the 2-fold higher prevalence of MGUS in African Americans vs whites.
  • Age-adjusted and standardized (to the 2000 world population) prevalence estimates of MGUS and 95% confidence intervals (CIs) were computed in the Ghanaian men and compared with MGUS prevalence in 7996 white men from Minnesota.
  • Associations between selected characteristics and MGUS prevalence were assessed by the Fisher exact test and logistic regression models.
  • RESULTS: Of the 917 study participants, 54 were found to have MGUS, yielding an age-adjusted prevalence of 5.84 (95% CI, 4.27-7.40) per 100 persons.
  • The concentration of monoclonal immunoglobulin was undetectable in 41 (76%) of the 54 MGUS cases, less than 1 g/dL in 10 patients (19%), and 1 g/dL or more in only 3 patients (6%).
  • Compared with white men, the age-adjusted prevalence of MGUS was 1.97-fold (95% CI, 1.94-2.00) higher in Ghanaian men.
  • CONCLUSION: The prevalence of MGUS in Ghanaian men was twice that in white men, supporting the hypothesis that race-related genetic susceptibility could explain the higher rates of MGUS in black populations.
  • An improved understanding of MGUS and MM pathophysiology would facilitate the development of strategies to prevent progression of MGUS to MM.
  • [MeSH-minor] Age Distribution. Aged. European Continental Ancestry Group / statistics & numerical data. Genetic Predisposition to Disease. Ghana / epidemiology. Humans. Male. Middle Aged. Minnesota / epidemiology. Prevalence. Socioeconomic Factors

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  • [CommentIn] Mayo Clin Proc. 2008 May;83(5):601-2; author reply 602-3 [18452695.001]
  • [CommentIn] Mayo Clin Proc. 2007 Dec;82(12):1457-9 [18053450.001]
  • (PMID = 18053453.001).
  • [ISSN] 0025-6196
  • [Journal-full-title] Mayo Clinic proceedings
  • [ISO-abbreviation] Mayo Clin. Proc.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA107-476-03; United States / NCI NIH HHS / CA / CA62242; United States / Intramural NIH HHS / /
  • [Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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72. Nageswara Rao AA, Rodriguez V, Long ME, Winters JL, Nichols WL, Pruthi RK: Transient neonatal acquired von Willebrand syndrome due to transplacental transfer of maternal monoclonal antibodies. Pediatr Blood Cancer; 2009 Oct;53(4):655-7
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  • [Title] Transient neonatal acquired von Willebrand syndrome due to transplacental transfer of maternal monoclonal antibodies.
  • Although typically a disorder of adults, acquired von Willebrand syndrome (AVWS) is increasingly being recognized in the pediatric population in association with congenital cardiac diseases, certain neoplasia, and hypothyroidism.
  • Transplacental transfer of maternal immunoglobulin G (IgG) antibodies as a cause of neonatal disorders in infants born to mothers with autoimmune conditions has been reported.
  • We describe the diagnosis and peripartum clinical management of AVWS due to monoclonal gammopathy of undetermined significance (MGUS) and the first reported case of transient neonatal AVWS due to transplacental transfer of maternal IgG antibodies.
  • [MeSH-major] Antibodies, Monoclonal / metabolism. Maternal-Fetal Exchange. von Willebrand Diseases / etiology

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  • (PMID = 19459202.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Immunoglobulin G
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73. Benson MD, Breall J, Cummings OW, Liepnieks JJ: Biochemical characterisation of amyloid by endomyocardial biopsy. Amyloid; 2009 Mar;16(1):9-14
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  • Three patients had monoclonal protein demonstrated in serum or urine and all three had bone marrow findings consistent with monoclonal gammopathy.
  • Seven patients had isolated cardiomyopathy without evidence of monoclonal gammopathy.
  • Two specimens obtained from patients with transthyretin (TTR) DNA mutations contained TTR peptides proving the hereditary nature of the disease.
  • Biopsies from four patients without a TTR mutation contained TTR and were consistent with the diagnosis of senile cardiac amyloidosis (SCA).
  • [MeSH-major] Amyloid / chemistry. Amyloidosis / diagnosis. Cardiomyopathy, Restrictive / metabolism
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Amino Acid Sequence. Biopsy. Female. Heart Failure / diagnosis. Humans. Male. Monoclonal Gammopathy of Undetermined Significance / metabolism. Prealbumin / genetics

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  • (PMID = 19291509.001).
  • [ISSN] 1744-2818
  • [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 / NIA NIH HHS / AG / AG 10133; United States / NIDDK NIH HHS / DK / DK 42111; United States / NCRR NIH HHS / RR / RR 00750
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Amyloid; 0 / Prealbumin
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74. Varella TC, Nishimura MY, Machado MC, de Moraes-Vasconcelos D, Rivitti EA: Schnitzler's syndrome without monoclonal gammopathy. Acta Derm Venereol; 2005;85(3):272-3
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  • [Title] Schnitzler's syndrome without monoclonal gammopathy.
  • [MeSH-major] Arthralgia / diagnosis. Immunoglobulin M
  • [MeSH-minor] Adult. Anti-Inflammatory Agents, Non-Steroidal / administration & dosage. Diagnosis, Differential. Drug Therapy, Combination. Humans. Male. Prednisone / administration & dosage. Syndrome. Thalidomide / administration & dosage. Urticaria / etiology. Urticaria / pathology

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  • (PMID = 16040423.001).
  • [ISSN] 0001-5555
  • [Journal-full-title] Acta dermato-venereologica
  • [ISO-abbreviation] Acta Derm. Venereol.
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] Norway
  • [Chemical-registry-number] 0 / Anti-Inflammatory Agents, Non-Steroidal; 0 / Immunoglobulin M; 4Z8R6ORS6L / Thalidomide; VB0R961HZT / Prednisone
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75. Bustany S, Gautier P, Lequerrec A, Troussard X, Ollivier Y, Borel-Derlon A: [Acquired von Willebrand syndrome: from diagnosis to treatment]. Pathol Biol (Paris); 2009 Nov-Dec;57(7-8):536-42
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  • [Title] [Acquired von Willebrand syndrome: from diagnosis to treatment].
  • [Transliterated title] Le syndrome de Willebrand acquis: du diagnostic au traitement.
  • Acquired von Willebrand syndrome is a rare bleeding disorder, which has been related in various diseases including lymphoproliferative disorders or autoimmune diseases.
  • Its diagnosis is an important step before treatment of patients and particularly in case of bleeding.
  • Mucocutaneous bleeds in every case were the same as in hereditary von Willebrand disease.
  • Phenotype was identified as type 2 von Willebrand disease with a loss of high molecular weight multimers.
  • The etiological diagnosis was one chronic lymphocytic leukaemia, two monoclonal gammapathies of undetermined significance (MGUS) and one undetermined case.
  • The management of patients need two stages: first infusions of factor von Willebrand/factor VIII concentrates to stop bleeds, then treatment of the underlying disease such as chemotherapy, corticotherapy and treatment with high doses of polyvalents immunoglobulins.
  • [MeSH-major] von Willebrand Disease, Type 2 / etiology

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  • (PMID = 19193498.001).
  • [ISSN] 1768-3114
  • [Journal-full-title] Pathologie-biologie
  • [ISO-abbreviation] Pathol. Biol.
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / von Willebrand Factor; 9001-27-8 / Factor VIII
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76. Bacovsky J, Scudla V, Myslivecek M, Nekula J, Vytrasová M: Scintigraphy using (99m)Tc-MIBI (sestamibi), a sensitive parameter of activity of multiple myeloma. Neoplasma; 2005;52(4):302-6
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  • In this study, 102 patients with multiple myeloma (MM) and 32 patients with monoclonal gammopathy of undetermined significance (MGUS) had been evaluated for correlation between (99m)Tc-MIBI and biochemical and hematological markers of activity of the disease.
  • Significant statistical correlation was found between summary score (SS) of (99m)Tc-MIBI scintigrams and beta2-microglobulin (p<0.001), monoclonal immunoglobulin level MIG (p<0.001), serum thymidinekinase - sTK (p<0.001), CRP (p<0.05) and cross-linked carboxyterminal telopeptide of type I collagen - ICTP (p<0.05) bone marrow plasmocytosis-BMPc (p<0.001) and hemoglobin Hb (p<0.001).
  • All 32 patients with MGUS had physiological activity of (99m)Tc-MIBI scintigrams.
  • Technetium-99m methoxyisobutylisonitrile is a useful indicator of activity of MM and helps in differentiating between multiple myeloma and monoclonal gammopathy of undetermined significance.
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Diagnosis, Differential. Disease Progression. Female. Humans. Male. Middle Aged. Radiopharmaceuticals. Technetium Tc 99m Sestamibi

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  • (PMID = 16059646.001).
  • [ISSN] 0028-2685
  • [Journal-full-title] Neoplasma
  • [ISO-abbreviation] Neoplasma
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Slovakia
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 971Z4W1S09 / Technetium Tc 99m Sestamibi
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77. Wechalekar AD, Offer M, Gillmore JD, Hawkins PN, Lachmann HJ: Cardiac amyloidosis, a monoclonal gammopathy and a potentially misleading mutation. Nat Clin Pract Cardiovasc Med; 2009 Feb;6(2):128-33
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  • [Title] Cardiac amyloidosis, a monoclonal gammopathy and a potentially misleading mutation.
  • DIAGNOSIS: Cardiac acquired monoclonal immunoglobulin-light-chain amyloidosis with the incidental presence of the amyloidogenic transthyretin Val122Ile mutation.
  • MANAGEMENT: The patient was referred for consideration of urgent cardiac transplantation and subsequent autologous stem cell transplantation.
  • [MeSH-major] Amyloidosis / diagnosis. Cardiomyopathy, Restrictive / diagnosis. Immunoglobulin kappa-Chains / analysis. Mutation. Paraproteinemias / diagnosis. Prealbumin / genetics

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  • [Cites] Circulation. 2005 Jan 18;111(2):186-93 [15630027.001]
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  • (PMID = 19079367.001).
  • [ISSN] 1743-4300
  • [Journal-full-title] Nature clinical practice. Cardiovascular medicine
  • [ISO-abbreviation] Nat Clin Pract Cardiovasc Med
  • [Language] eng
  • [Grant] United Kingdom / Medical Research Council / / G7900510; United Kingdom / Medical Research Council / / G97900510
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Immunoglobulin kappa-Chains; 0 / Prealbumin
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78. Hamidou MA, Belizna C, Wiertlewsky S, Audrain M, Biron C, Grolleau JY, Mussini JM: Intravenous cyclophosphamide in refractory polyneuropathy associated with IgM monoclonal gammopathy: an uncontrolled open trial. Am J Med; 2005 Apr;118(4):426-30
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  • [Title] Intravenous cyclophosphamide in refractory polyneuropathy associated with IgM monoclonal gammopathy: an uncontrolled open trial.

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  • (PMID = 15808143.001).
  • [ISSN] 0002-9343
  • [Journal-full-title] The American journal of medicine
  • [ISO-abbreviation] Am. J. Med.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunoglobulin M; 8N3DW7272P / Cyclophosphamide
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79. Kyle RA, Benson J, Larson D, Therneau T, Dispenzieri A, Melton Iii LJ, Rajkumar SV: IgM monoclonal gammopathy of undetermined significance and smoldering Waldenström's macroglobulinemia. Clin Lymphoma Myeloma; 2009 Mar;9(1):17-8
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  • [Title] IgM monoclonal gammopathy of undetermined significance and smoldering Waldenström's macroglobulinemia.
  • Immunoglobulin M monoclonal gammopathy of undetermined significance (IgM-MUS) was diagnosed in 213 Mayo Clinic patients who were residents of 11 counties in southeastern Minnesota from 1960 to 1994.
  • During long-term follow-up, 29 (14%) developed non-Hodgkin lymphoma (n = 17), Waldenström's macroglobulinemia (WM; n = 6), chronic lymphocytic leukemia (n = 3), and AL amyloidosis (n = 3) with relative risks of 15-, 262-, 6-, and 16-fold, respectively.
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Disease Progression. Female. Follow-Up Studies. Humans. Male. Middle Aged. Minnesota / epidemiology. Multivariate Analysis. Young Adult

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  • (PMID = 19362962.001).
  • [ISSN] 1557-9190
  • [Journal-full-title] Clinical lymphoma & myeloma
  • [ISO-abbreviation] Clin Lymphoma Myeloma
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / R01 CA107476
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunoglobulin M
  • [Other-IDs] NLM/ NIHMS498185; NLM/ PMC3773469
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80. Karakelides M, Monson KL, Volcheck GW, Weiler CR: Monoclonal gammopathies and malignancies in patients with chronic urticaria. Int J Dermatol; 2006 Sep;45(9):1032-8
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  • [Title] Monoclonal gammopathies and malignancies in patients with chronic urticaria.
  • BACKGROUND: Monoclonal gammopathy of undetermined significance (MGUS) has been described in association with chronic urticaria (CU) in patients with Schnitzler syndrome.
  • METHODS: This study was conducted to evaluate the frequency and characteristics of MGUS or malignancy in patients with CU.
  • The Mayo Clinic electronic database was reviewed to identify patients with the diagnosis of CU.
  • RESULTS: Of the 1639 patients presenting with CU between 1994 and 2001, 797 (49%) underwent laboratory evaluation for the presence of a coexisting monoclonal protein.
  • Forty-seven CU patients had MGUS, 142 had a malignancy, and 24 had both.
  • Fifteen percent of CU patients with MGUS had a hematologic malignancy compared with 0.9% of CU patients without MGUS (P < 0.001).
  • Patients presenting with a new diagnosis of CU at an older age (> 56 years) were more likely to have associated underlying MGUS.
  • The occurrence of MGUS in this group was higher than the reported incidence of MGUS in the general population.
  • CONCLUSIONS: Patients with CU younger than 43 years were unlikely to have associated MGUS or malignancy.
  • A higher percentage of patients with CU and MGUS had an associated diagnosis of hematologic malignancy.
  • [MeSH-minor] Adult. Age Distribution. Age Factors. Aged. Blood Proteins / metabolism. Chronic Disease. Complement C4 / metabolism. Female. Humans. Immunoglobulin A / blood. Immunoglobulin G / blood. Immunoglobulin M / blood. Male. Middle Aged. Thyrotropin / blood

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  • (PMID = 16961504.001).
  • [ISSN] 0011-9059
  • [Journal-full-title] International journal of dermatology
  • [ISO-abbreviation] Int. J. Dermatol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Blood Proteins; 0 / Complement C4; 0 / Immunoglobulin A; 0 / Immunoglobulin G; 0 / Immunoglobulin M; 9002-71-5 / Thyrotropin
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81. Vital C, Lagueny A, Mercie P, Viallard JF, Delabrousse-Mayoux JP, Vital A: Usefulness of combined nerve and muscle biopsy in the diagnosis of amyloid neuropathy--a study of 6 new cases. Clin Neuropathol; 2010 Mar-Apr;29(2):59-64
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  • [Title] Usefulness of combined nerve and muscle biopsy in the diagnosis of amyloid neuropathy--a study of 6 new cases.
  • There were 2 patients with an idiopathic polyneuropathy and 4 with monoclonal gammopathy (MG).
  • [MeSH-major] Amyloid Neuropathies / diagnosis. Muscle, Skeletal / pathology. Peroneal Nerve / pathology


82. Hoyer C, Angermann CE, Knop S, Ertl G, Störk S: [Cardiac amyloidosis]. Med Klin (Munich); 2008 Mar 15;103(3):153-60
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  • There can also be chest pain, probably due to microvessel disease.
  • It can be performed by monoclonal gammopathy.
  • The desirable treatment therapy consists of high-dose melphalan therapy twice followed by autologous stem cell transplantation.
  • The ATTR amyloidosis is an autosomal dominant disorder caused by the amyloidogenic form of transthyretin, a plasmaprotein that is synthesized in the liver.
  • [MeSH-minor] Adult. Aged. Amyloidosis, Familial / diagnosis. Amyloidosis, Familial / genetics. Biopsy. Echocardiography. Female. Humans. Immunoglobulin Light Chains. Magnetic Resonance Imaging. Male. Melphalan / administration & dosage. Melphalan / therapeutic use. Myocardium / pathology. Practice Guidelines as Topic. Prognosis. Stem Cell Transplantation. Transplantation, Autologous

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  • (PMID = 18344065.001).
  • [ISSN] 0723-5003
  • [Journal-full-title] Medizinische Klinik (Munich, Germany : 1983)
  • [ISO-abbreviation] Med. Klin. (Munich)
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Immunoglobulin Light Chains; Q41OR9510P / Melphalan
  • [Number-of-references] 21
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83. Saad T, Agmon-Levin N, Shoenfeld Y: [Chronic stimulation of the immune system in sarcoidosis and monoclonal gammopathy of undetermined significance]. Harefuah; 2009 Dec;148(12):809-10, 857, 856
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  • [Title] [Chronic stimulation of the immune system in sarcoidosis and monoclonal gammopathy of undetermined significance].
  • Sarcoidosis is a systemic granulomatous disease which predominantly involves the lungs.
  • Monoclonal gammopathy of undetermined significance (MGUS) characterized by clonal proliferation of plasma cells, can develop into multiple myeloma at a later stage.
  • Although the cause of sarcoidosis remains unknown, the most likely etiology relates to chronic stimulation of the immune system that may result in polyclonal B cell proliferation.
  • In some patients this polyclonal proliferation may develop into a monoclonal B-cell proliferation and induce the appearance of monoclonal gammopathy.
  • In this article, a possible linkage between sarcoidosis and MGUS is raised, based on the case study of a 67-year-old woman and a review of the literature.

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  • (PMID = 20088430.001).
  • [ISSN] 0017-7768
  • [Journal-full-title] Harefuah
  • [ISO-abbreviation] Harefuah
  • [Language] heb
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Israel
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84. Rojas-Garcia R, Gallardo E, De La Torre C, Sanvito L, Illa I: Chronic sensorimotor polyradiculopathy with antibodies to P2: an electrophysiological and immunoproteomic analysis. Muscle Nerve; 2008 Jul;38(1):933-8
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  • In this study we report a patient with chronic progressive sensory ataxia, proximal weakness, immunoglobulin M (IgM) monoclonal gammopathy, and elevated protein levels in the cerebrospinal fluid, who showed a good response to prednisone.
  • [MeSH-minor] Aged. Anti-Inflammatory Agents / therapeutic use. Blotting, Western. Chronic Disease. Electrophoresis, Gel, Two-Dimensional. Electrophysiology. Enzyme-Linked Immunosorbent Assay. Humans. Male. Nerve Tissue Proteins / biosynthesis. Nerve Tissue Proteins / genetics. Prednisone / therapeutic use. Spinal Nerve Roots / pathology

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  • [Copyright] (c) 2008 Wiley Periodicals, Inc.
  • (PMID = 18508345.001).
  • [ISSN] 0148-639X
  • [Journal-full-title] Muscle & nerve
  • [ISO-abbreviation] Muscle Nerve
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Anti-Inflammatory Agents; 0 / Myelin P2 Protein; 0 / Nerve Tissue Proteins; VB0R961HZT / Prednisone
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86. de Alba Campomanes AG, Rutar T, Crawford JB, Seiff S, Goodman D, Grenert J: Crystal-storing histiocytosis and crystalline keratopathy caused by monoclonal gammopathy of undetermined significance. Cornea; 2009 Oct;28(9):1081-4
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  • [Title] Crystal-storing histiocytosis and crystalline keratopathy caused by monoclonal gammopathy of undetermined significance.
  • PURPOSE: The purpose of this study was to report the occurrence of crystalline keratopathy and of orbital infiltrative disease resulting from crystal-storing histiocytosis (CSH) in a patient with monoclonal gammopathy of undetermined significance.
  • METHODS: The authors conducted a review of a medical record and immunohistopathologic studies.
  • The systemic evaluation was consistent with monoclonal gammopathy of undetermined significance.
  • For unknown reasons, in this patient, a systemic immunologic disorder led to lambda light chain abnormalities with histiocyte infiltration of the orbit and corneal deposition.
  • Although CSH is rare, it should be part of the differential diagnosis of orbital infiltrative disease with or without crystalline keratopathy.
  • [MeSH-major] Corneal Diseases / etiology. Histiocytosis / etiology. Monoclonal Gammopathy of Undetermined Significance / complications

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  • (PMID = 19724196.001).
  • [ISSN] 1536-4798
  • [Journal-full-title] Cornea
  • [ISO-abbreviation] Cornea
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunoglobulin lambda-Chains
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87. Harati A, Brockmeyer NH, Altmeyer P, Kreuter A: Skin disorders in association with monoclonal gammopathies. Eur J Med Res; 2005 Mar 29;10(3):93-104
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  • [Title] Skin disorders in association with monoclonal gammopathies.
  • Monoclonal gammopathy represents a condition characterized by clonal proliferation and accumulation of immunoglobulin producing B-cells.
  • A variety of skin disorders are associated with an increased level of monoclonal immunoglobulin proteins.
  • The first group represents a direct consequence of plasma cell proliferation.
  • The colonization of the plasma cell clone in the dermis expressed as a deposition of proteins related to the M component belongs to this group for which the pathogenesis is well identified, as is the case for example with AL amyloidosis and cryoglobulins.
  • The second group represents skin disorders such as scleromyxedema and Schnitzler syndrome that are highly associated with an M component, or diseases such as pyoderma gangrenosum and leukocytoclastic vasculitis that are more weakly associated with increased levels of monoclonal immunoglobulins.
  • In some other dermatoses such as pemphigus, bullous pemphigoid, epidermolysis bullosa aquisita, Sezary syndrome, lymphomatoid papulosis, urticaria pigmentosa, and acquired ichthyosis, only presumptions exist regarding associations with monoclonal gammopathies.
  • In this the pathogenesis, therapy and prognosis of the most relevant dermatoses shall be described in order of their degree of association with monoclonal gammopathies, which shall also be discussed.

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  • (PMID = 15851375.001).
  • [ISSN] 0949-2321
  • [Journal-full-title] European journal of medical research
  • [ISO-abbreviation] Eur. J. Med. Res.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 86
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88. Brenne AT, Fagerli UM, Shaughnessy JD Jr, Våtsveen TK, Rø TB, Hella H, Zhan F, Barlogie B, Sundan A, Børset M, Waage A: High expression of BCL3 in human myeloma cells is associated with increased proliferation and inferior prognosis. Eur J Haematol; 2009 May;82(5):354-63
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  • We experienced that this putative oncogene was a common target gene for growth-promoting cytokines in myeloma cell lines.
  • METHODS: Gene expression of BCL3 was studied in 351 newly diagnosed myeloma patients, 12 patients with smouldering myeloma, 44 patients with monoclonal gammopathy of undetermined significance and 22 healthy individuals.
  • A total of eight different myeloma cell lines were studied.
  • RESULTS: Bcl-3 was induced in myeloma cell lines by interleukin (IL)-6, IL-21, IL-15, tumor necrosis factor-alpha and IGF-1, and its upregulation was associated with increased proliferation of the cells.
  • When this patient population was divided into subgroups based on molecular classification, BCL3 was significantly increased in a poor risk subgroup characterized by overexpression of cell cycle and proliferation related genes.
  • Intracellular localization of Bcl-3 was dependent on type of stimulus given to the cell.
  • CONCLUSION: BCL3 is a common target gene for several growth-promoting cytokines in myeloma cells and high expression of BCL3 at the time of diagnosis is associated with poor prognosis of patients with multiple myeloma (MM).

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  • (PMID = 19191868.001).
  • [ISSN] 1600-0609
  • [Journal-full-title] European journal of haematology
  • [ISO-abbreviation] Eur. J. Haematol.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / P01 CA055819; United States / NCI NIH HHS / CA / R33 CA097513
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Cytokines; 0 / Proto-Oncogene Proteins; 0 / RNA, Messenger; 0 / Transcription Factors; 0 / proto-oncogene protein bcl-3
  • [Other-IDs] NLM/ PMC2704939
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89. Lee HY, Yoo SM, Song IS, Yu H, Lee JB, Shin JW, Park IW: A case of nonsecretory multiple myeloma with atypical imaging features. Korean J Intern Med; 2006 Sep;21(3):202-5
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  • Multiple myeloma usually shows homogeneous enhancement on contrast-enhanced Magnetic Resonance imaging (MRI), and is accompanied by a monoclonal gammopathy in serum or urine.
  • We report a case of nonsecretory myeloma, the diagnosis was difficult due to the absence of a monoclonal gammopathy and the presence of atypical imaging features.
  • [MeSH-major] Bone Marrow / pathology. Multiple Myeloma / diagnosis. Spinal Neoplasms / diagnosis. Thoracic Vertebrae / pathology
  • [MeSH-minor] Diagnosis, Differential. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Tomography, Emission-Computed

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  • (PMID = 17017673.001).
  • [ISSN] 1226-3303
  • [Journal-full-title] The Korean journal of internal medicine
  • [ISO-abbreviation] Korean J. Intern. Med.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Korea (South)
  • [Other-IDs] NLM/ PMC3890727
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90. Bladé J, Rosiñol L, Cibeira MT, de Larrea CF: Pathogenesis and progression of monoclonal gammopathy of undetermined significance. Leukemia; 2008 Sep;22(9):1651-7
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  • [Title] Pathogenesis and progression of monoclonal gammopathy of undetermined significance.
  • In Caucasians, monoclonal gammopathy of undetermined significance (MGUS) is an age-related condition with prevalence as high as 3% in persons older than 50 years.
  • Compared with whites, blacks have around two- and threefold higher prevalence rates of MGUS and multiple myeloma (MM), respectively.
  • Risk of progression from MGUS to MM has been found to be very similar in whites and blacks.
  • On average, the transformation rate to a malignant monoclonal gammopathy is 1% per year, with the mechanisms of progression likely related to bone marrow microenvironment and/or the cytokine network.
  • The predictors of malignant transformation are the plasma cell mass (M-protein size and/or proportion of plasma cell in the bone marrow), IgA isotype, serum free light-chain ratio and 'evolving' type and ratio between phenotypically aberrant and normal bone marrow plasma cells.
  • [MeSH-major] Monoclonal Gammopathy of Undetermined Significance / etiology. Multiple Myeloma / etiology
  • [MeSH-minor] Cell Transformation, Neoplastic / pathology. Diagnosis, Differential. Disease Progression. Humans

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  • (PMID = 18668131.001).
  • [ISSN] 1476-5551
  • [Journal-full-title] Leukemia
  • [ISO-abbreviation] Leukemia
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] England
  • [Number-of-references] 63
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91. Shimizu K, Itoh J, Sugiura I, Tsushita K, Kosugi H, Nagura E: [Evaluation of the clinical relevance of serum measurements of free-light chains in patients with multiple myeloma]. Rinsho Ketsueki; 2006 Apr;47(4):303-9
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  • Measurement of serum free-light chains (FLCs) was performed using a recently developed immunoassay in 180 healthy individuals, 16 patients with multiple myeloma, and each 1 patient with Waldenström's macroglobulinemia, primary amyloidosis, or MGUS (monoclonal gammopathy of undetermined significance) to evaluate the clinical relevance of FLCs in the diagnosis and disease monitoring.
  • The serum FLC assay is a sensitive and useful tool for the diagnosis and monitoring of multiple myeloma and other B-cell proliferative disorders.

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  • (PMID = 16715965.001).
  • [ISSN] 0485-1439
  • [Journal-full-title] [Rinshō ketsueki] The Japanese journal of clinical hematology
  • [ISO-abbreviation] Rinsho Ketsueki
  • [Language] jpn
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Immunoglobulin Light Chains; 0 / Immunoglobulin kappa-Chains; 0 / Immunoglobulin lambda-Chains
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92. Bernez A, Abdallah-Lotf M, D'Incan M, De Muret A, Souteyrand P, Lorette G, Machet L: [Necrobiotic xanthogranuloma without monoclonal gammopathy and with a rapidly fatal outcome]. Ann Dermatol Venereol; 2006 Mar;133(3):246-9
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  • [Title] [Necrobiotic xanthogranuloma without monoclonal gammopathy and with a rapidly fatal outcome].
  • [Transliterated title] Xanthogranulome nécrobiotique sans gammapathie monoclonale d'évolution rapidement fatale.
  • Benign monoclonal gammopathy associated with myeloma is found in 80% of patients, but the course is normally long, with 100% survival at 10 years.
  • The clinical appearance and histopathological examination resulted in diagnosis of necrobiotic xanthogranuloma.
  • No monoclonal gammopathy or myeloma was seen.
  • The disease was marked by sensitivity to corticosteroids with failure of other therapies (cyclophosphamide, alpha interferon), onset ofcorticosteroid dependency, iatrogenic Cushing's syndrome and diabetes, which were in part responsible for the infectious complications and subsequent death of the patient.
  • DISCUSSION: Necrobiotic xanthogranuloma is difficult to treat, even in the absence of myeloma or monoclonal gammopathy.
  • [MeSH-major] Granuloma / diagnosis. Necrobiotic Disorders / diagnosis. Xanthomatosis / diagnosis

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  • (PMID = 16800175.001).
  • [ISSN] 0151-9638
  • [Journal-full-title] Annales de dermatologie et de vénéréologie
  • [ISO-abbreviation] Ann Dermatol Venereol
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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93. Machaczka M, Kalaitzakis E, Eleborg L, Ljungman P, Hägglund H: Comparison of general vs regional anaesthesia for BM harvesting: a retrospective study of anaesthesia-related complications. Bone Marrow Transplant; 2010 Jan;45(1):53-61
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  • This retrospective study was conducted to evaluate the safety and complications profile of general anaesthesia (GA) compared with that of regional anaesthesia (RA) for BM harvesting (BMH).
  • However, the incidence of postoperative events was higher in the allogeneic group compared with that in the autologous group (25 vs 10%, P<0.01) and in female donors compared with male donors (29 vs 14%, P=0.002).
  • In conclusion, both GA and RA are comparable with regard to BMH.
  • Nevertheless, non-severe intra- and postoperative events were frequent.

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  • (PMID = 19483763.001).
  • [ISSN] 1476-5365
  • [Journal-full-title] Bone marrow transplantation
  • [ISO-abbreviation] Bone Marrow Transplant.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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94. Chatterjee M, Jain S, Stühmer T, Andrulis M, Ungethüm U, Kuban RJ, Lorentz H, Bommert K, Topp M, Krämer D, Müller-Hermelink HK, Einsele H, Greiner A, Bargou RC: STAT3 and MAPK signaling maintain overexpression of heat shock proteins 90alpha and beta in multiple myeloma cells, which critically contribute to tumor-cell survival. Blood; 2007 Jan 15;109(2):720-8
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  • [Title] STAT3 and MAPK signaling maintain overexpression of heat shock proteins 90alpha and beta in multiple myeloma cells, which critically contribute to tumor-cell survival.
  • Given the importance of the BMM for drug resistance and MM-cell survival, apoptosis induced by Hsp90 inhibition was not mitigated in the presence of bone marrow stromal cells, osteoclasts, or endothelial cells.
  • Finally, in situ overexpression of both Hsp90 proteins was observed in most MMs but not in monoclonal gammopathy of undetermined significance (MGUS) or in normal plasma cells.
  • [MeSH-minor] Apoptosis / drug effects. Benzoquinones / pharmacology. Cell Line. Cell Survival / drug effects. Coculture Techniques. Down-Regulation / drug effects. Down-Regulation / immunology. Humans. Lactams, Macrocyclic / pharmacology. MAP Kinase Signaling System. Phosphorylation. RNA Interference. RNA, Small Interfering / pharmacology. Signal Transduction. Structure-Activity Relationship

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  • [ReprintIn] Verh Dtsch Ges Pathol. 2007;91:330-7 [18314631.001]
  • (PMID = 17003370.001).
  • [ISSN] 0006-4971
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Benzoquinones; 0 / HSP90 Heat-Shock Proteins; 0 / Lactams, Macrocyclic; 0 / RNA, Small Interfering; 0 / STAT3 Transcription Factor; 0 / STAT3 protein, human; 001L2FE0M3 / 17-(dimethylaminoethylamino)-17-demethoxygeldanamycin; EC 2.7.11.24 / Mitogen-Activated Protein Kinases
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95. Petrillo G, Catalano L, Petruzziello F, Padula S, Petrillo C, Caso P: [Amyloid cardiomyopathy: a link between cardiology and hematology. A case report of positive response to standard therapy]. G Ital Cardiol (Rome); 2007 Jun;8(6):371-6
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  • [Transliterated title] La cardiomiopatia amiloidotica: un'interfaccia fra cardiologia ed ematologia. Descrizione di un caso clinico con risposta favorevole alla terapia tradizionale.
  • We report the case of a 56-year-old woman who came to our observation because of symptoms of congestive heart failure.
  • Diagnosis of restrictive cardiomyopathy was made by echocardiographic examination, which showed right ventricular hypertrophy, disarray of interventricular septum and restrictive flow pattern at the mitral valve.
  • Laboratory findings confirmed biopsy results, leading to the definite diagnosis of restrictive cardiomyopathy due to IgA kappa monoclonal gammopathy in primary systemic amyloidosis.
  • [MeSH-major] Amyloidosis / complications. Amyloidosis / diagnosis. Cardiomyopathy, Restrictive / complications. Immunoglobulin kappa-Chains / metabolism

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  • [CommentIn] G Ital Cardiol (Rome). 2007 Jun;8(6):377-80 [17633912.001]
  • (PMID = 17633911.001).
  • [ISSN] 1827-6806
  • [Journal-full-title] Giornale italiano di cardiologia (2006)
  • [ISO-abbreviation] G Ital Cardiol (Rome)
  • [Language] ita
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Immunoglobulin kappa-Chains
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96. Zappasodi P, Corso A, Klersy C, Pica G, Mangiacavalli S, Varettoni M, Rusconi C, Pascutto C, Lazzarino M: Changes in multiple myeloma epidemiology in the last thirty years: a single centre experience. Eur J Cancer; 2006 Feb;42(3):396-402
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  • Patients were divided, according to the date of diagnosis in group I or group II (before and after 1994, respectively) and therapy (high or conventional dose).
  • Bone pain and early deaths were statistically reduced in group II, whereas MM that evolved from monoclonal gammopathy of undetermined significance (MGUS) had increased.
  • The new therapeutic approaches, chemotherapy and supportive therapy, allowed better control of the disease with an improvement of survival.
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Female. Humans. Italy / epidemiology. Male. Middle Aged. Neoplasm Recurrence, Local / mortality. Neoplasm Staging / methods. Survival Analysis

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  • (PMID = 16403623.001).
  • [ISSN] 0959-8049
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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97. Jaskowski TD, Litwin CM, Hill HR: Detection of kappa and lambda light chain monoclonal proteins in human serum: automated immunoassay versus immunofixation electrophoresis. Clin Vaccine Immunol; 2006 Feb;13(2):277-80
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  • [Title] Detection of kappa and lambda light chain monoclonal proteins in human serum: automated immunoassay versus immunofixation electrophoresis.
  • Recently, turbidimetric immunoassays for detecting and quantifying kappa and lambda free light chains (FLC) have become available and are promoted as being more sensitive than immunofixation electrophoresis (IFE) in detecting FLC monoclonal proteins.
  • In this study, we assessed the ability of these turbidimetric assays to detect serum monoclonal proteins involving both free and heavy-chain-bound kappa and lambda light chains compared to standard immunofixation electrophoresis.
  • Sera demonstrating a restricted band of protein migration (other than a definite M spike) by serum protein electrophoresis (SPE), which may represent early monoclonal proteins, were also examined.
  • When compared to IFE, percent agreement, sensitivity, and specificity for the kappa-FLC and lambda-FLC were 94.6, 72.9, and 99.5% and 98.5, 91.4, and 99.7%, respectively, in detecting monoclonal proteins involving free and heavy-chain-bound light chains.
  • The majority of sera (73.7%) demonstrating a restricted band of protein migration on SPE demonstrated abnormal IFE patterns suggestive of multiple myeloma or monoclonal gammopathy of unknown significance, but gave normal kappa/lambda FLC ratios using the turbidimetric immunoassays.
  • In conclusion, the kappa and lambda FLC assays are significantly less sensitive (72.9 to 91.4%) than IFE, but specific in detecting serum monoclonal proteins.
  • [MeSH-major] Antibodies, Monoclonal / blood. Immunoassay / methods. Immunoelectrophoresis / methods. Immunoglobulin kappa-Chains / blood. Immunoglobulin lambda-Chains / blood
  • [MeSH-minor] Blood Protein Electrophoresis / methods. Blood Protein Electrophoresis / statistics & numerical data. Humans. Monoclonal Gammopathy of Undetermined Significance / immunology. Multiple Myeloma / immunology. Nephelometry and Turbidimetry / methods. Nephelometry and Turbidimetry / statistics & numerical data. Sensitivity and Specificity

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  • (PMID = 16467338.001).
  • [ISSN] 1556-6811
  • [Journal-full-title] Clinical and vaccine immunology : CVI
  • [ISO-abbreviation] Clin. Vaccine Immunol.
  • [Language] eng
  • [Publication-type] Comparative Study; Evaluation Studies; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Immunoglobulin kappa-Chains; 0 / Immunoglobulin lambda-Chains
  • [Other-IDs] NLM/ PMC1391945
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98. Kristinsson SY, Fears TR, Gridley G, Turesson I, Mellqvist UH, Björkholm M, Landgren O: Deep vein thrombosis after monoclonal gammopathy of undetermined significance and multiple myeloma. Blood; 2008 Nov 1;112(9):3582-6
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  • [Title] Deep vein thrombosis after monoclonal gammopathy of undetermined significance and multiple myeloma.
  • Recently, 2 small hospital-based studies observed persons with the MM precursor condition, monoclonal gammopathy of undetermined significance (MGUS), to be at increased risk of developing DVT.
  • Among 4 196 197 veterans hospitalized at least once at US Veterans Affairs hospitals, we identified a total of 2374 cases of MGUS, and 39 272 persons were diagnosed with DVT (crude incidence 0.9 per 1000 person-years).
  • A total of 31 and 151 DVTs occurred among MGUS and MM patients, respectively (crude incidence 3.1 and 8.7 per 1000 person-years, respectively; P < .01).
  • Compared with the entire study population, the relative risk (RR) of DVT after a diagnosis of MGUS and MM was 3.3 (95% confidence interval [CI], 2.3-4.7) and 9.2 (95% CI, 7.9-10.8), respectively.
  • The most prominent excess risk of DVT was found during the first year after diagnosis of MGUS (RR = 8.4; 95% CI, 5.7-12.2) and MM (RR = 11.6; 95% CI, 9.2-14.5).
  • Among 229 MGUS cases (9.5%) that progressed to MM, only one person had a DVT diagnosis before transformation.
  • Our findings suggest the operation of shared underlying mechanisms causing coagulation abnormalities among patients with MGUS and MM.

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  • (PMID = 18559977.001).
  • [ISSN] 1528-0020
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] ENG
  • [Grant] United States / Intramural NIH HHS / /
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC2572787
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99. Peest D, Ganser A: [Therapy of multiple myeloma: indications and options]. Internist (Berl); 2007 Dec;48(12):1343-8
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  • MM has to be distinguished from smouldering MM and monoclonal gammopathy of uncertain significance (MGUS).
  • In younger patients (<65 years) a good long-term remission is the aim of therapy, while in the elderly patients with comorbidities the aim is a good partial remission with good quality of life.
  • High-dose chemotherapy, often as a tandem transplantation, is part of standard therapy of MM patients <65 years.
  • However, allogeneic stem cell transplantation is the only curative approach.
  • [MeSH-minor] Boronic Acids / administration & dosage. Boronic Acids / adverse effects. Bortezomib. Combined Modality Therapy. Diphosphonates / administration & dosage. Diphosphonates / adverse effects. Dose-Response Relationship, Drug. Hematopoietic Stem Cell Transplantation. Humans. Melphalan / administration & dosage. Melphalan / adverse effects. Neoplasm Staging. Prednisone / administration & dosage. Prednisone / adverse effects. Pyrazines / administration & dosage. Pyrazines / adverse effects. Remission Induction. Thalidomide / administration & dosage. Thalidomide / adverse effects. Thalidomide / analogs & derivatives. Tumor Burden

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  • Hazardous Substances Data Bank. MELPHALAN .
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  • (PMID = 17960351.001).
  • [ISSN] 0020-9554
  • [Journal-full-title] Der Internist
  • [ISO-abbreviation] Internist (Berl)
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Boronic Acids; 0 / Diphosphonates; 0 / Pyrazines; 4Z8R6ORS6L / Thalidomide; 69G8BD63PP / Bortezomib; F0P408N6V4 / lenalidomide; Q41OR9510P / Melphalan; VB0R961HZT / Prednisone
  • [Number-of-references] 26
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100. Adam Z, Bolcak K, Stanicek J, Buchler T, Pour L, Krejci M, Prasek J, Neubauer J, Vorlicek J, Hajek R: Fluorodeoxyglucose positron emission tomography in multiple myeloma, solitary plasmocytoma and monoclonal gammapathy of unknown significance. Neoplasma; 2007;54(6):536-40
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  • [Title] Fluorodeoxyglucose positron emission tomography in multiple myeloma, solitary plasmocytoma and monoclonal gammapathy of unknown significance.
  • The aim of our study was to evaluate the role of fluorine-18 fluorodeoxyglucose positron emission tomography (FDGPET) in 49 patients with plasma cell malignancies.
  • Of the 20 patients who had negative FDG-PET scans, only one relapsed 12 months after FDG-PET examination.. FDG-PET was positive in two of six patients with MGUS and with suspected progression to MM or with suspected other malignancy.
  • We conclude that FDG PET might contribute to initial staging of MM patients with negative bone radiographs and is useful for the follow-up of patients in remission especially in non-secretory MM and in patients with large plasmocytoma (>5 cm) after radiochemotherapy.
  • [MeSH-major] Fluorodeoxyglucose F18. Multiple Myeloma / diagnosis. Paraproteinemias / diagnosis. Plasmacytoma / diagnosis. Positron-Emission Tomography
  • [MeSH-minor] Adult. Aged. Female. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Neoplasm Recurrence, Local / pathology. Neoplasm Staging. Tomography, Emission-Computed

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  • (PMID = 17949238.001).
  • [ISSN] 0028-2685
  • [Journal-full-title] Neoplasma
  • [ISO-abbreviation] Neoplasma
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] Slovakia
  • [Chemical-registry-number] 0Z5B2CJX4D / Fluorodeoxyglucose F18
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