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1. Franco V, Florena AM, Iannitto E: Splenic marginal zone lymphoma. Blood; 2003 Apr 1;101(7):2464-72
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  • [Title] Splenic marginal zone lymphoma.
  • Splenic marginal zone lymphoma (SMZL) is a specific low-grade small B-cell lymphoma that is incorporated in the World Health Organization classification.
  • Characteristic features are splenomegaly, moderate lymphocytosis with villous morphology, intrasinusoidal pattern of involvement of various organs, especially bone marrow, and relative indolent course.
  • To date, no definitive therapy has been established.
  • Therapeutic options include treatment abstention, splenectomy, splenic irradiation, and chemotherapy.
  • [MeSH-major] Lymphoma, B-Cell / pathology. Lymphoma, Non-Hodgkin / pathology. Splenic Neoplasms / pathology
  • [MeSH-minor] Animals. Cytogenetic Analysis. Diagnosis, Differential. Humans. Immunophenotyping

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  • (PMID = 12446449.001).
  • [ISSN] 0006-4971
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Number-of-references] 144
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2. Chacón JI, Mollejo M, Muñoz E, Algara P, Mateo M, Lopez L, Andrade J, Carbonero IG, Martínez B, Piris MA, Cruz MA: Splenic marginal zone lymphoma: clinical characteristics and prognostic factors in a series of 60 patients. Blood; 2002 Sep 1;100(5):1648-54
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Splenic marginal zone lymphoma: clinical characteristics and prognostic factors in a series of 60 patients.
  • A precise description of clinical features at presentation and analysis of clinical and biologic prognostic factors in splenic marginal zone lymphoma (SMZL) are still lacking.
  • All 60 patients received splenectomies, 29 of 60 also received chemotherapy, and 2 received spleen radiotherapy.
  • At 5 years from diagnosis, 39 patients (65%) were alive.
  • Significant prognostic factors in multivariate analysis were (1) (for OS and FFS) lack of response to therapy (CR versus noncomplete response [nCR]) and involvement of nonhematopoietic sites, and (2) (for the probability of obtaining CR) bone marrow involvement.
  • Chemotherapy did not influence OS or FFS. p53 overexpression predicted a shorter OS in the univariate analysis.
  • These data confirm the relative indolence of this disease, indicating the existence of a subset of more aggressive cases, which should stimulate the search for predictive biologic factors and alternative therapies.
  • [MeSH-major] Lymphoma, B-Cell. Splenic Neoplasms

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  • (PMID = 12176884.001).
  • [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
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3. Szynglarewicz B, Matkowski R, Smorag Z, Forgacz J, Pudelko M, Kornafel J: Hepatitis C virus infection and locally advanced splenic marginal zone lymphoma. Pathol Oncol Res; 2007;13(4):382-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Hepatitis C virus infection and locally advanced splenic marginal zone lymphoma.
  • Splenic marginal zone lymphoma (SMZL) is a rare malignant B-cell neoplasm, usually with an indolent clinical course and favorable prognosis.
  • Treatment options include chemotherapy, surgery, radiation and immunotherapy.
  • Neither peripheral lymphadenopathy nor bone marrow pathology was found.
  • Following surgery, chemotherapy (CHOP regimen) and immunotherapy (anti-CD20 antibody) were given.
  • At the last follow-up 15 months after surgery, the patient was free of any symptoms of lymphoma.
  • Surgical resection of even locally advanced SMZL with involvement of adjacent tissues can be performed as a diagnostic and therapeutic procedure.
  • HCV infection may result in increased risk of SMZL due to the induction of B-cell lymphoproliferation.
  • Because of possible lymphoma regression following anti-viral therapy, a systematic screening for HCV in patients with SMZL seems to be valuable and helpful for treatment planning.
  • [MeSH-major] Hepatitis C / complications. Lymphoma, B-Cell, Marginal Zone / diagnosis. Lymphoma, B-Cell, Marginal Zone / virology. Splenic Neoplasms / diagnosis. Splenic Neoplasms / virology

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  • [Cites] Cancer. 2004 Nov 1;101(9):2050-7 [15389479.001]
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  • (PMID = 18158578.001).
  • [ISSN] 1219-4956
  • [Journal-full-title] Pathology oncology research : POR
  • [ISO-abbreviation] Pathol. Oncol. Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
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4. Thieblemont C, Felman P, Berger F, Dumontet C, Arnaud P, Hequet O, Arcache J, Callet-Bauchu E, Salles G, Coiffier B: Treatment of splenic marginal zone B-cell lymphoma: an analysis of 81 patients. Clin Lymphoma; 2002 Jun;3(1):41-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Treatment of splenic marginal zone B-cell lymphoma: an analysis of 81 patients.
  • Splenic marginal zone B-cell lymphoma (MZL), with or without villous lymphocytes, is an indolent lymphoma, presenting with massive splenomegaly, generally associated with bone marrow dissemination.
  • In patients requiring therapy, splenectomy has been reported as the treatment of choice.
  • We reviewed the cases of 81 patients with splenic MZL.
  • Patients presented with stage IV disease at diagnosis in 95% of the cases.
  • Twenty patients did not receive any initial treatment at diagnosis.
  • Splenectomy was proposed in 79% of the treated patients, with adjuvant chemotherapy in 47% of patients.
  • Seventy percent of the patients had persistent involvement of bone marrow and/or peripheral blood after splenectomy.
  • Disease progression was significantly more frequent in partial responders than in complete responders (P < 0.005), but overall survival, risk of histologic transformation, and risk of death from lymphoma were not different in the 2 groups.
  • Moreover, patients with cytopenia at diagnosis treated by splenectomy alone rapidly recovered normal hematological parameters.
  • We conclude that splenectomy is an efficient treatment for splenic MZL, but that it may be delayed until the occurrence of symptoms or cytopenia.
  • [MeSH-major] Lymphoma, B-Cell / surgery. Splenectomy. Splenic Neoplasms / surgery. Vidarabine / analogs & derivatives
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Antibodies, Monoclonal / blood. Antineoplastic Agents / therapeutic use. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Autoimmune Diseases / etiology. Bleomycin / administration & dosage. Bone Marrow / pathology. Bone Marrow Diseases / etiology. Chemotherapy, Adjuvant. Chlorambucil / therapeutic use. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Disease Progression. Doxorubicin / administration & dosage. Female. Follow-Up Studies. Humans. Lymphoma, B-Cell, Marginal Zone / complications. Lymphoma, B-Cell, Marginal Zone / drug therapy. Lymphoma, B-Cell, Marginal Zone / pathology. Lymphoma, B-Cell, Marginal Zone / surgery. Male. Middle Aged. Paraproteins / analysis. Prednisone / administration & dosage. Prognosis. Remission Induction. Retrospective Studies. Survival Analysis. Treatment Outcome. Vincristine / administration & dosage

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  • [CommentIn] Clin Lymphoma. 2002 Jun;3(1):48 [12141955.001]
  • (PMID = 12141954.001).
  • [ISSN] 1526-9655
  • [Journal-full-title] Clinical lymphoma
  • [ISO-abbreviation] Clin Lymphoma
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antineoplastic Agents; 0 / Paraproteins; 11056-06-7 / Bleomycin; 18D0SL7309 / Chlorambucil; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; FA2DM6879K / Vidarabine; P2K93U8740 / fludarabine; VB0R961HZT / Prednisone; ACVB protocol; CHOP protocol
  • [Number-of-references] 17
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5. Franco V, Florena AM, Stella M, Rizzo A, Iannitto E, Quintini G, Campesi G: Splenectomy influences bone marrow infiltration in patients with splenic marginal zone cell lymphoma with or without villous lymphocytes. Cancer; 2001 Jan 15;91(2):294-301
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Splenectomy influences bone marrow infiltration in patients with splenic marginal zone cell lymphoma with or without villous lymphocytes.
  • BACKGROUND: Splenic marginal zone cell lymphoma (SMZL) is a low grade B-cell lymphoma in which patients can have circulating villous lymphocytes and can show a peculiar intrasinusoidal bone marrow (BM) infiltration.
  • Splenectomy is the reported treatment of choice for these patients.
  • RESULTS: BM infiltration ranged from 10% to 40% of overall cellularity and was mostly of the intrasinusoidal type.
  • These effects suggest reconsidering the role of splenectomy in the treatment of patients with SMZL.
  • [MeSH-major] Bone Marrow / pathology. Lymphoma, B-Cell / pathology. Lymphoma, Non-Hodgkin / pathology. Splenectomy
  • [MeSH-minor] Adult. Aged. Female. Humans. Lymphocytosis / drug therapy. Lymphocytosis / etiology. Male. Middle Aged

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  • (PMID = 11180074.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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6. Elmahy H, Hawley I, Beard J: Composite splenic marginal zone lymphoma and classic Hodgkin lymphoma -- an unusual combination. Int J Lab Hematol; 2007 Dec;29(6):461-3
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  • [Title] Composite splenic marginal zone lymphoma and classic Hodgkin lymphoma -- an unusual combination.
  • The simultaneous occurrence of Hodgkin lymphoma with a variety of B-cell Non-Hodgkin lymphomas (composite lymphoma) has been described.
  • We report the first case of composite Hodgkin lymphoma and splenic marginal zone lymphoma occurring simultaneously in the same lymph node of a 64-year-old man who presented with cervical and axillary lymphadenopathy and massive splenomegaly.
  • He had a peripheral blood lymphocytosis and a bone marrow infiltrated by small lymphocytes.
  • However, cervical lymph node biopsy showed classic Hodgkin lymphoma.
  • His splenomegaly showed only a partial response to six cycles of ABVD chemotherapy so he underwent splenectomy with biopsy of remaining nodes.
  • Histology of the spleen and nodes showed splenic marginal zone lymphoma.
  • [MeSH-major] Hodgkin Disease / pathology. Lymphoma, B-Cell, Marginal Zone / pathology. Neoplasms, Second Primary / pathology. Splenic Neoplasms / pathology
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Biopsy. Bleomycin / administration & dosage. Bone Marrow Cells / pathology. Dacarbazine / administration & dosage. Doxorubicin / administration & dosage. Humans. Lymph Nodes / pathology. Lymphocytes / pathology. Lymphocytosis / pathology. Lymphocytosis / therapy. Male. Middle Aged. Splenectomy. Splenomegaly / pathology. Splenomegaly / therapy. Vinblastine / administration & dosage

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  • (PMID = 17988302.001).
  • [ISSN] 1751-5521
  • [Journal-full-title] International journal of laboratory hematology
  • [ISO-abbreviation] Int J Lab Hematol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 11056-06-7 / Bleomycin; 5V9KLZ54CY / Vinblastine; 7GR28W0FJI / Dacarbazine; 80168379AG / Doxorubicin; ABVD protocol
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7. Phanish MK, Owen A, Parry DH: Spontaneous regression of acquired C1 esterase inhibitor deficiency associated with splenic marginal zone lymphoma presenting with recurrent angio-oedema. J Clin Pathol; 2002 Oct;55(10):789-90

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Spontaneous regression of acquired C1 esterase inhibitor deficiency associated with splenic marginal zone lymphoma presenting with recurrent angio-oedema.
  • A 52 year old woman with marginal zone lymphoma developed recurrent episodes of angio-oedema and was found to have C1 esterase inhibitor deficiency.
  • She declined chemotherapy for the lymphoma.
  • Fourteen months after her initial presentation she was found to be in partial remission, and this was confirmed by peripheral blood film and bone marrow examinations.
  • Regression of acquired C1 esterase inhibitor deficiency associated with spontaneous partial remission of lymphoma has not been reported previously.
  • [MeSH-major] Angioedema / etiology. Complement C1 Inactivator Proteins / deficiency. Lymphoma, B-Cell / complications. Paraneoplastic Syndromes / etiology. Splenic Neoplasms / complications

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  • (PMID = 12354812.001).
  • [ISSN] 0021-9746
  • [Journal-full-title] Journal of clinical pathology
  • [ISO-abbreviation] J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Complement C1 Inactivator Proteins
  • [Other-IDs] NLM/ PMC1769784
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8. Brown JR, Friedberg JW, Feng Y, Scofield S, Phillips K, Dal Cin P, Joyce R, Takvorian RW, Fisher DC, Fisher RI, Liesveld J, Marquis D, Neuberg D, Freedman AS: A phase 2 study of concurrent fludarabine and rituximab for the treatment of marginal zone lymphomas. Br J Haematol; 2009 Jun;145(6):741-8
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  • [Title] A phase 2 study of concurrent fludarabine and rituximab for the treatment of marginal zone lymphomas.
  • The marginal zone lymphomas (MZLs) are a recently defined group of related diseases that probably arise from a common cell of origin, the marginal zone B cell.
  • Data on therapy for subtypes other than gastric mucosa-associated lymphoid tissue (MALT) lymphoma has been largely limited to retrospective case series.
  • This prospective phase 2 study of fludarabine and rituximab for the treatment of marginal zone lymphomas enrolled 26 patients, 14 with nodal MZL, eight with MALT lymphomas and four with splenic MZL; 81% were receiving initial systemic therapy.
  • Four late toxic deaths occurred due to infections [15% (95% CI 4.3-35%)], two related to delayed bone marrow aplasia and two related to myelodysplastic syndrome.
  • We conclude that, although concurrent fludarabine and rituximab given at this dose and schedule is a highly effective regimen in the treatment of MZLs, the significant haematological and infectious toxicity observed both during and after therapy is prohibitive in this patient population, emphasizing the need to study MZLs as a separate entity.

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  • [CommentIn] Br J Haematol. 2009 Sep;146(6):685-6 [19638019.001]
  • (PMID = 19344412.001).
  • [ISSN] 1365-2141
  • [Journal-full-title] British journal of haematology
  • [ISO-abbreviation] Br. J. Haematol.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / K23 CA115682-03; United States / NCI NIH HHS / CA / K23 CA115682-04; None / None / / K23 CA115682-03; United States / NCI NIH HHS / CA / CA115682-01; United States / NCI NIH HHS / CA / P01 CA092625; United States / NCI NIH HHS / CA / R21 CA103244; United States / NCI NIH HHS / CA / K23 CA115682; United States / NCI NIH HHS / CA / P50CA130805; United States / NCI NIH HHS / CA / CA115682-04; United States / NCI NIH HHS / CA / K23 CA115682-01; United States / NCI NIH HHS / CA / CA-103244; United States / NCI NIH HHS / CA / CA115682-02; United States / NCI NIH HHS / CA / P50 CA130805; United States / NCI NIH HHS / CA / 2P01CA092625; United States / NCI NIH HHS / CA / K23 CA115682-02
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Multicenter Study; Research Support, N.I.H., Extramural
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Murine-Derived; 4F4X42SYQ6 / Rituximab; FA2DM6879K / Vidarabine; P2K93U8740 / fludarabine
  • [Other-IDs] NLM/ NIHMS110464; NLM/ PMC2752822
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9. Audouin J, Le Tourneau A, Molina T, Camilleri-Broët S, Adida C, Comperat E, Benattar L, Delmer A, Devidas A, Rio B, Diebold J: Patterns of bone marrow involvement in 58 patients presenting primary splenic marginal zone lymphoma with or without circulating villous lymphocytes. Br J Haematol; 2003 Aug;122(3):404-12
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Patterns of bone marrow involvement in 58 patients presenting primary splenic marginal zone lymphoma with or without circulating villous lymphocytes.
  • We studied 86 bone marrow biopsies (BMB) from 58 patients presenting with primary splenic marginal zone lymphoma (PSMZL).
  • In 42 patients, a splenectomy was performed which enabled a histopathological diagnosis.
  • In 16 recently observed patients, 17 BMB led to PSMZL diagnosis, and these patients were treated without splenectomy.
  • Seven different patterns of infiltrates were recognized: intravascular, interstitial, nodular, massive, plasmacytic mimicking myeloma and transformation into large B-cell lymphoma (DLBCL).
  • The association of an intravascular infiltrate and nodules with a germinal centre and/or a marginal zone favoured a diagnosis of MZL.
  • Immunohistochemistry demonstrated the expression of B cell-associated antigens and, in 40% of the patients, a monotypic lymphoplasmacytic cell component.
  • In the past, such cases have been diagnosed as lymphoplasmacytic lymphoma.
  • Successive biopsies showed progression and, after chemotherapy, a slight decrease in infiltrates.
  • The patterns described are not specific for PSMZL and occur also in primary nodal MZL and, more rarely, in MALT-type lymphoma.
  • [MeSH-major] B-Lymphocytes / pathology. Bone Marrow Cells / pathology. Leukemic Infiltration. Lymphoma / pathology
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Bone Marrow Examination. Diagnosis, Differential. Disease Progression. Female. Humans. Immunohistochemistry. Lymphoma, Large B-Cell, Diffuse / pathology. Male. Middle Aged. Plasma Cells / pathology. Splenectomy. Waldenstrom Macroglobulinemia / diagnosis

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  • [CommentIn] Br J Haematol. 2004 Jan;124(2):252-3 [14687039.001]
  • (PMID = 12877667.001).
  • [ISSN] 0007-1048
  • [Journal-full-title] British journal of haematology
  • [ISO-abbreviation] Br. J. Haematol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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10. Bruna J, Martínez-Yelamos S, Alonso E, Romagosa V, Arruga J, Arruga J, Domingo A, Rojas-Marcos I, Petit J, Rubio F: Meningeal lymphomatosis as the first manifestation of splenic marginal zone lymphoma. Int J Hematol; 2005 Jul;82(1):63-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Meningeal lymphomatosis as the first manifestation of splenic marginal zone lymphoma.
  • Meningeal lymphomatosis (ML) as the first manifestation of a splenic marginal zone lymphoma (SMZL) is rare.
  • The bone marrow section also showed lymphocytes with an immunophenotype identical to that of the peripheral blood.A splenectomy confirmed the SMZL diagnosis.
  • Treatment with corticosteroids and intrathecal chemotherapy was administrated; however, the response was not good, and the patient died.
  • In this report, we discuss the other 2 cases and ML in B-cell chronic lymphoproliferative disorders.
  • [MeSH-major] Lymphoma / complications. Lymphoproliferative Disorders / etiology. Meninges / pathology. Splenic Neoplasms / complications
  • [MeSH-minor] Adrenal Cortex Hormones / therapeutic use. Antigens, CD / analysis. Fatal Outcome. Humans. Male. Middle Aged

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  • (PMID = 16105762.001).
  • [ISSN] 0925-5710
  • [Journal-full-title] International journal of hematology
  • [ISO-abbreviation] Int. J. Hematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Adrenal Cortex Hormones; 0 / Antigens, CD
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11. Bennett M, Schechter GP: Treatment of splenic marginal zone lymphoma: splenectomy versus rituximab. Semin Hematol; 2010 Apr;47(2):143-7
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  • [Title] Treatment of splenic marginal zone lymphoma: splenectomy versus rituximab.
  • Splenic marginal zone lymphoma (SMZL) is an uncommon indolent B-cell lymphoma causing marked splenic enlargement with CD20-rich lymphoma cells infiltrating blood and bone marrow.
  • In the pre-rituximab era, the treatment of choice for patients with symptomatic splenomegaly or threatening cytopenia was splenectomy, since chemotherapy had limited efficacy.
  • Since approval of rituximab, treatment of such patients with the anti-CD20 antibody both alone or in combination with chemotherapy has shown remarkable responses.
  • In retrospective series of rituximab monotherapy totaling 52 patients, including both chemotherapy-naive and -refractory patients, overall responses of 88% to 100% were noted with marked and prompt regression of splenomegaly and improvement of cytopenias.
  • These results suggest that splenectomy should no longer be considered as initial therapy for SMZL but rather as palliative therapy for patients not responsive to immunotherapy with or without chemotherapy.
  • [MeSH-major] Antibodies, Monoclonal / therapeutic use. Antineoplastic Agents / therapeutic use. Lymphoma, B-Cell, Marginal Zone / therapy. Splenic Neoplasms / therapy
  • [MeSH-minor] Antibodies, Monoclonal, Murine-Derived. Antigens, CD20 / analysis. Antigens, CD20 / immunology. Antigens, CD79 / analysis. Antigens, Neoplasm / analysis. Antimetabolites, Antineoplastic / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Clinical Trials as Topic / statistics & numerical data. Combined Modality Therapy. Disease-Free Survival. Follow-Up Studies. Humans. Immunophenotyping. Palliative Care. Remission Induction. Rituximab. Splenomegaly / etiology. Splenomegaly / radiotherapy. Splenomegaly / surgery. Survival Analysis. Treatment Outcome

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  • (PMID = 20350661.001).
  • [ISSN] 1532-8686
  • [Journal-full-title] Seminars in hematology
  • [ISO-abbreviation] Semin. Hematol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Murine-Derived; 0 / Antigens, CD20; 0 / Antigens, CD79; 0 / Antigens, Neoplasm; 0 / Antimetabolites, Antineoplastic; 0 / Antineoplastic Agents; 0 / CD79B protein, human; 4F4X42SYQ6 / Rituximab
  • [Number-of-references] 31
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12. Fabbri A, Gozzetti A, Lazzi S, Lenoci M, D'Amuri A, Leoncini L, Lauria F: Activity of rituximab monotherapy in refractory splenic marginal zone lymphoma complicated with autoimmune hemolytic anemia. Clin Lymphoma Myeloma; 2006 May;6(6):496-9
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  • [Title] Activity of rituximab monotherapy in refractory splenic marginal zone lymphoma complicated with autoimmune hemolytic anemia.
  • We describe the case of a 61-year-old patient with refractory splenic marginal zone lymphoma and secondary autoimmune hemolytic anemia, both successfully treated with rituximab.
  • This case demonstrates that rituximab monotherapy might also be a valid therapeutic approach in marginal zone lymphoma and autoimmune hemolytic anemia after failure of first-line treatment.
  • Maintenance therapy, although expensive, could be useful to improve event-free survival in patients with unfavorable clinical behavior.
  • [MeSH-major] Anemia, Hemolytic, Autoimmune / complications. Antibodies, Monoclonal / therapeutic use. Antineoplastic Agents / therapeutic use. Lymphoma, B-Cell / complications. Lymphoma, B-Cell / drug therapy
  • [MeSH-minor] Antibodies, Monoclonal, Murine-Derived. Biopsy. Bone Marrow / pathology. Humans. Male. Middle Aged. Prednisone / therapeutic use. Rituximab. Splenectomy. Treatment Outcome

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  • (PMID = 16796783.001).
  • [ISSN] 1557-9190
  • [Journal-full-title] Clinical lymphoma & myeloma
  • [ISO-abbreviation] Clin Lymphoma Myeloma
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Murine-Derived; 0 / Antineoplastic Agents; 4F4X42SYQ6 / Rituximab; VB0R961HZT / Prednisone
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13. Chang ST, Hsieh YC, Lu YH, Tzeng CC, Lin CN, Chuang SS: Floral leukemic cells transformed from marginal zone lymphoma. Pathol Res Pract; 2008;204(1):23-6

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Floral leukemic cells transformed from marginal zone lymphoma.
  • There are three clinicopathological entities of marginal zone lymphoma (MZL), including extranodal or mucosa-associated lymphoid tissue (MALT) lymphoma and MZL of nodal (NMZL) or splenic (SMZL) type.
  • We present a stage 4 MZL involving lymph node, spleen, and bone marrow with two relapses after chemotherapy.
  • The leukemic cells at the second relapse revealed irregular nuclear contours with multilobated nuclei (so-called flower cells or floral cells) mimicking the neoplastic cells in adult T-cell leukemia/lymphoma (ATLL).
  • The absence of leukemic change and splenic hilar lymphadenopathy at initial presentation, expression of IgD by tumor cells, and cytogenetic changes of +7 suggested that this tumor might be a NMZL.
  • Although the cytomorphologic features of floral leukemic cells might suggest ATLL, thorough clinical and laboratory workup helped to reach a correct diagnosis.
  • [MeSH-major] Bone Marrow Cells / pathology. Leukemia-Lymphoma, Adult T-Cell / diagnosis. Lymph Nodes / pathology. Lymphocytes / pathology. Lymphoma, B-Cell, Marginal Zone / diagnosis. Spleen / pathology
  • [MeSH-minor] Aged. Antineoplastic Agents / therapeutic use. Cell Nucleus / pathology. Cytogenetics. Diagnosis, Differential. Fatal Outcome. Gene Expression Regulation, Neoplastic. Humans. Immunoglobulin D / analysis. Male. Radiotherapy. Recurrence. Treatment Failure

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  • (PMID = 17913385.001).
  • [ISSN] 0344-0338
  • [Journal-full-title] Pathology, research and practice
  • [ISO-abbreviation] Pathol. Res. Pract.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Immunoglobulin D
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14. Ou J, Yang L, Ren L, Tang X, Li T, Wu S: [The clinical features and tumor cells characteristics of splenic marginal zone lymphoma]. Zhonghua Nei Ke Za Zhi; 2002 Jan;41(1):28-30
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  • [Title] [The clinical features and tumor cells characteristics of splenic marginal zone lymphoma].
  • OBJECTIVE: To deepen the understanding of splenic marginal zone lymphoma (SMZL) and improve the level of diagnosis and therapy.
  • The pathologic, immunologic and genetic features of tumor cells in peripheral blood, bone marrow and spleen were studied with light microscopy, phase contrast microscopy, scanning electron microscopy, immunohistochemical method, flow cytometry, G chromosome banding technique and PCR for studying the pattern of IgH gene rearrangement.
  • Histologically, the neoplastic cells replaced the marginal and mantle zones with complete replacement of germinal centers in the white pulp.
  • Lymph nodes in the splenic hilum were infiltrated by tumor cells.
  • Immunophenotypic analysis demonstrated that the lymphocytes in the bone marrow expressed CD(20), HLA-DR, CD(45) RA and bcl-2.
  • The monoclonal pattern of IgH gene rearrangement in peripheral blood and bone marrow was found to be the same as that in spleen.
  • After splenectomy, COP chemotherapy and IFNalpha-2a were given and the abnormally increased lymphocytes decreased to normal level.
  • CONCLUSION: Splenomegaly, lymphocytosis in peripheral blood and bone marrow without lymph node enlargement and leukocytosis are clinical characters of SMZL.
  • Presence of monoclonal rearranged IgH gene is in favor of the diagnosis.
  • [MeSH-major] Germinal Center / pathology. Lymphoma, B-Cell / pathology. Splenic Neoplasms / pathology
  • [MeSH-minor] Antigens, CD20 / biosynthesis. Antigens, CD45 / biosynthesis. Cyclophosphamide / therapeutic use. Female. Gene Rearrangement. HLA-DR Antigens / biosynthesis. Humans. Immunoglobulin Heavy Chains / genetics. Middle Aged. Proto-Oncogene Proteins c-bcl-2 / biosynthesis. Splenectomy. Splenomegaly / pathology

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  • (PMID = 11940293.001).
  • [ISSN] 0578-1426
  • [Journal-full-title] Zhonghua nei ke za zhi
  • [ISO-abbreviation] Zhonghua Nei Ke Za Zhi
  • [Language] chi
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Antigens, CD20; 0 / HLA-DR Antigens; 0 / Immunoglobulin Heavy Chains; 0 / Proto-Oncogene Proteins c-bcl-2; 8N3DW7272P / Cyclophosphamide; EC 3.1.3.48 / Antigens, CD45
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15. Dungarwalla M, Appiah-Cubi S, Kulkarni S, Saso R, Wotherspoon A, Osuji N, Swansbury J, Cunningham DC, Catovsky D, Dearden CE, Matutes E: High-grade transformation in splenic marginal zone lymphoma with circulating villous lymphocytes: the site of transformation influences response to therapy and prognosis. Br J Haematol; 2008 Oct;143(1):71-4
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  • [Title] High-grade transformation in splenic marginal zone lymphoma with circulating villous lymphocytes: the site of transformation influences response to therapy and prognosis.
  • In a series of 48 patients with splenic marginal zone lymphoma (SMZL) with circulating villous lymphocytes, we describe the clinical and laboratory features of nine cases that transformed to high-grade B-cell lymphoma.
  • These patients had a significantly greater incidence of peripheral lymph node involvement at diagnosis when compared to SMZL patients who did not transform (P < 0.03).
  • While transformation in the bone marrow is frequently refractory to therapy and associated with poor outcome in SMZL, lymph node transformation responds well to chemotherapy with durable progression-free and overall survival.
  • [MeSH-major] Cell Transformation, Neoplastic / pathology. Lymph Nodes / pathology. Lymphocytes / pathology. Lymphoma, B-Cell, Marginal Zone / pathology. Splenic Neoplasms / pathology

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  • (PMID = 18671706.001).
  • [ISSN] 1365-2141
  • [Journal-full-title] British journal of haematology
  • [ISO-abbreviation] Br. J. Haematol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] England
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16. Harada S, Kalla H, Balasubramanian M, Brodsky I, Gladstone D, Hou JS: Classical Hodgkin lymphoma concurrently evolving in a patient with marginal zone B-cell lymphoma of the spleen. Ann Diagn Pathol; 2008 Jun;12(3):212-6
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  • [Title] Classical Hodgkin lymphoma concurrently evolving in a patient with marginal zone B-cell lymphoma of the spleen.
  • Combination of the splenic marginal zone B-cell lymphoma (SMZL) and classical Hodgkin lymphoma (cHL) is extremely rare.
  • A splenectomy revealed monotonous marginal zone lymphocytic infiltrates and numerous large Reed-Sternberg-like cells.
  • Flow cytometry revealed a kappa light-chain-restricted CD5 (-), CD23 (-) B-cell population.
  • After the chemotherapy, the patient achieved a clinical/radiologic remission, whereas cHL was detected in liver and bone marrow subsequently.
  • The case indicates that both components of lymphoma can present concurrently as a composite form of lymphoma and both need to be treated adequately.
  • [MeSH-major] Hodgkin Disease / pathology. Lymphoma, B-Cell, Marginal Zone / pathology. Neoplasms, Second Primary / pathology. Splenic Neoplasms / pathology
  • [MeSH-minor] Antigens, CD / analysis. B-Lymphocytes / chemistry. B-Lymphocytes / pathology. Combined Modality Therapy. Gene Rearrangement, B-Lymphocyte, Heavy Chain / genetics. Humans. Lymphatic Diseases. Male. Middle Aged. Reed-Sternberg Cells / pathology. Remission Induction. Splenomegaly

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  • (PMID = 18486899.001).
  • [ISSN] 1532-8198
  • [Journal-full-title] Annals of diagnostic pathology
  • [ISO-abbreviation] Ann Diagn Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD
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17. Matutes E, Oscier D, Montalban C, Berger F, Callet-Bauchu E, Dogan A, Felman P, Franco V, Iannitto E, Mollejo M, Papadaki T, Remstein ED, Salar A, Solé F, Stamatopoulos K, Thieblemont C, Traverse-Glehen A, Wotherspoon A, Coiffier B, Piris MA: Splenic marginal zone lymphoma proposals for a revision of diagnostic, staging and therapeutic criteria. Leukemia; 2008 Mar;22(3):487-95
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  • [Title] Splenic marginal zone lymphoma proposals for a revision of diagnostic, staging and therapeutic criteria.
  • Since the initial description of splenic marginal zone lymphoma (SMZL) in 1992, an increasing number of publications have dealt with multiple aspects of SMZL diagnosis, molecular pathogenesis and treatment.
  • This process has identified multiple inconsistencies in the diagnostic criteria and lack of clear guidelines for the staging and treatment.
  • The authors of this review have held several meetings and exchanged series of cases with the objective of agreeing on the main diagnostic, staging and therapeutic guidelines for patients with this condition.
  • Specific working groups were created for diagnostic criteria, immunophenotype, staging and treatment.
  • As results of this work, guidelines are proposed for diagnosis, differential diagnosis, staging, prognostic factors, treatment and response criteria.
  • The guidelines proposed here are intended to contribute to the standardization of the diagnosis and treatment of these patients, and should facilitate the future development of clinical trials that could define more precisely predictive markers for histological progression or lack of response, and evaluate new drugs or treatments.
  • [MeSH-major] Lymphoma, B-Cell, Marginal Zone. Splenic Neoplasms
  • [MeSH-minor] Antibodies, Monoclonal / therapeutic use. Antibodies, Monoclonal, Murine-Derived. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Antiviral Agents / therapeutic use. Biomarkers, Tumor / blood. Bone Marrow / pathology. Chromosome Aberrations. Combined Modality Therapy. Comorbidity. Diagnosis, Differential. Disease Management. Hepatitis C, Chronic / complications. Hepatitis C, Chronic / drug therapy. Humans. Immunophenotyping. Neoplasm Staging / methods. Neoplasm Staging / standards. Practice Guidelines as Topic. Prognosis. Rituximab. Spleen / pathology. Splenectomy

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  • (PMID = 18094718.001).
  • [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
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Murine-Derived; 0 / Antiviral Agents; 0 / Biomarkers, Tumor; 4F4X42SYQ6 / Rituximab
  • [Number-of-references] 81
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18. Traverse-Glehen A, Felman P, Callet-Bauchu E, Gazzo S, Baseggio L, Bryon PA, Thieblemont C, Coiffier B, Salles G, Berger F: A clinicopathological study of nodal marginal zone B-cell lymphoma. A report on 21 cases. Histopathology; 2006 Jan;48(2):162-73

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A clinicopathological study of nodal marginal zone B-cell lymphoma. A report on 21 cases.
  • AIMS: To report the clinicopathological findings of 21 cases of primary nodal marginal zone B-cell lymphoma (NMZL).
  • NMZL is a recently characterized lymphoma and few series have been published.
  • METHODS AND RESULTS: The clinical data were characteristic of a disseminated disease at presentation: presence of peripheral and abdominal lymph nodes, bone marrow involvement (62%), disease stage III and IV (76%), elevated lactate dehydrogenase (LDH) (48%).
  • Relapses were frequent but the majority of patients receiving chemotherapy had a good initial response.
  • Morphological features were heterogeneous and there were some differences compared with other marginal zone B-cell lymphomas (MZL).
  • Pure monocytoid B-cell lymphomas were rare (10%) but a minor component of monocytoid B cell was observed more frequently (23%).
  • CONCLUSION: NMZL can be distinguished from splenic MZL and extranodal MZL by its aggressive morphology and disseminated disease at presentation.
  • [MeSH-major] Lymphoma, B-Cell / pathology
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Antigens, CD20 / analysis. Bone Marrow / pathology. DNA-Binding Proteins / analysis. Female. Gene Rearrangement, B-Lymphocyte, Heavy Chain / genetics. Humans. Immunohistochemistry. Karyotyping. Lymph Nodes / pathology. Male. Middle Aged. Neoplasm Staging. Proto-Oncogene Proteins c-bcl-2 / analysis. Survival Analysis. Translocation, Genetic

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  • (PMID = 16405665.001).
  • [ISSN] 0309-0167
  • [Journal-full-title] Histopathology
  • [ISO-abbreviation] Histopathology
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, CD20; 0 / BCL6 protein, human; 0 / DNA-Binding Proteins; 0 / Proto-Oncogene Proteins c-bcl-2
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19. Svoboda J, Andreadis C, Downs LH, Miller Jr WT, Tsai DE, Schuster SJ: Regression of advanced non-splenic marginal zone lymphoma after treatment of hepatitis C virus infection. Leuk Lymphoma; 2005 Sep;46(9):1365-8
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  • [Title] Regression of advanced non-splenic marginal zone lymphoma after treatment of hepatitis C virus infection.
  • We describe a patient with chronic hepatitis C virus (HCV) infection who presented with extranodal (right salivary gland and bone marrow) marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT).
  • Within a year, the lymphoma progressed to involve peripheral lymph nodes and the liver.
  • After treatment of HCV with peginterferon and ribavirin, he achieved complete radiographic response and remains in remission two years later.
  • This is an example of a patient in whom treatment of HCV infection led to regression of non-splenic, extranodal marginal zone lymphoma.
  • It suggests that HCV testing and treatment should be considered in all patients with marginal zone lymphomas, including non-splenic types.
  • [MeSH-major] Hepatitis C / complications. Hepatitis C / drug therapy. Interferons / therapeutic use. Lymphoma, B-Cell, Marginal Zone / complications. Ribavirin / therapeutic use. Salivary Gland Neoplasms / complications
  • [MeSH-minor] Drug Therapy, Combination. Humans. Liver Neoplasms / secondary. Lymphatic Metastasis. Male. Middle Aged

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  • (PMID = 16109616.001).
  • [ISSN] 1042-8194
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 49717AWG6K / Ribavirin; 9008-11-1 / Interferons
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20. Parry-Jones N, Matutes E, Gruszka-Westwood AM, Swansbury GJ, Wotherspoon AC, Catovsky D: Prognostic features of splenic lymphoma with villous lymphocytes: a report on 129 patients. Br J Haematol; 2003 Mar;120(5):759-64

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Prognostic features of splenic lymphoma with villous lymphocytes: a report on 129 patients.
  • Splenic lymphoma with villous lymphocytes (SLVL) is a low-grade B-cell lymphoma defined in the World Health Organization classification as the leukaemic form of splenic marginal zone lymphoma.
  • Presenting features and response to therapy have been described, but information on prognostic factors is scanty.
  • Diagnosis was made on clinical, morphological and immunophenotypic features and, where available, bone marrow and spleen histology.
  • Median Hb was 11.8 g/dl, white blood cell count was 16 x 10(9)/l and platelet count was 145 x 10(9)/l; 27% of patients had monoclonal protein in serum and/or urine.
  • While 27% of patients remained untreated, 10% transformed to high-grade lymphoma.
  • However, only anaemia and lymphocytosis > 16 x 10(9)/l remained highly significant independent prognostic factors when only deaths due to lymphoma were analysed.
  • Splenectomized patients fared better than those receiving chemotherapy only (P = 0.001 for SLVL deaths).
  • We conclude that SLVL is mainly a disease of the elderly with a relatively benign course but, when treatment is required, splenectomy is beneficial.
  • [MeSH-major] B-Lymphocytes / pathology. Lymphoma, B-Cell / surgery. Splenic Neoplasms / surgery
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Female. Humans. Male. Middle Aged. Splenectomy. Survival Analysis. Treatment Outcome

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  • [CommentIn] Br J Haematol. 2003 Oct;123(2):370-1 [14531926.001]
  • (PMID = 12614206.001).
  • [ISSN] 0007-1048
  • [Journal-full-title] British journal of haematology
  • [ISO-abbreviation] Br. J. Haematol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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21. Carr JA, Shurafa M, Velanovich V: Surgical indications in idiopathic splenomegaly. Arch Surg; 2002 Jan;137(1):64-8
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  • HYPOTHESIS: The incidence of primary lymphoma of the spleen in patients with idiopathic splenomegaly is significant.
  • Of these, 18 had idiopathic splenomegaly despite prior workup with computed tomography, peripheral smear, bone marrow biopsy, and laboratory testing.
  • INTERVENTION: All 18 patients underwent open splenectomy for diagnosis and treatment of their cytopenias.
  • MAIN OUTCOME MEASURE: Incidence of lymphoma in the pathologic specimens.
  • In all 18 patients, the surgical specimen provided a diagnosis.
  • Six patients with the benign diagnosis of hypersplenism received no further interventions, and the cytopenias resolved in all 6 cases.
  • The 7 remaining patients (39%) were diagnosed with lymphoma.
  • Five had marginal zone lymphoma, and 2 had a more aggressive B-cell lymphoma.
  • Three patients required chemotherapy, but 4 are still in remission since their splenectomies and show no evidence of active disease.
  • CONCLUSIONS: A high percentage of patients with splenomegaly of unknown etiology will have primary lymphoma of the spleen.
  • Splenectomy is both diagnostic and therapeutic and should be considered for all patients with idiopathic splenomegaly.
  • [MeSH-major] Lymphoma, Non-Hodgkin / epidemiology. Splenic Neoplasms / epidemiology. Splenomegaly / surgery

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  • (PMID = 11772218.001).
  • [ISSN] 0004-0010
  • [Journal-full-title] Archives of surgery (Chicago, Ill. : 1960)
  • [ISO-abbreviation] Arch Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  •  go-up   go-down


22. Hamblin TJ: Achieving optimal outcomes in chronic lymphocytic leukaemia. Drugs; 2001;61(5):593-611
NCI CPTC Antibody Characterization Program. NCI CPTC Antibody Characterization Program .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • It needs to be distinguished from mantle cell lymphoma and splenic marginal zone lymphoma by lymphocyte markers.
  • The disease is staged according to the presence of lymphadenopathy and/or splenomegaly and the features of bone marrow suppression.
  • This group of patients have a normal life expectancy and do not require treatment beyond reassurance.
  • Progression involves an increasing white cell count, enlarging lymph nodes and spleen, anaemia and thrombocytopenia.
  • Complications of progression include autoimmune haemolytic anaemia and thrombocytopenia, immunodeficiency, and the development of a more aggressive lymphoma.
  • Intermittent chlorambucil remains the first choice treatment for the majority of patients.
  • Combination chemotherapy offers no advantage.
  • Intravenous fludarabine is probably more effective than chlorambucil, but no trial has yet shown a survival advantage for using it first rather than as a salvage treatment in patients not responding to chlorambucil.
  • It is at least 40 times as expensive as chlorambucil.
  • For patients refractory to both drugs, a variety of options are available.
  • High dose corticosteroids, high dose chlorambucil, CHOP (cyclophosphamide, prednisolone, vincristine and doxorubicin), anti-CD52, anti-CD20 and a range of experimental drugs which are being evaluated in clinical trials.
  • Younger patients should be offered the chance of treatment with curative intent, preferably in the context of a clinical trial.
  • Autologous stem cell transplantation after achieving a remission with fludarabine has relative safety and may produce molecular complete remissions.
  • Only time will tell whether some of these patients are cured but it seems unlikely.
  • Standard allogeneic bone marrow transplant is probably too hazardous for most patients, but non-myeloablative regimens hold out the hope of invoking a graft-versus-leukaemia effect without a high tumour-related mortality.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Leukemia, Lymphocytic, Chronic, B-Cell / drug therapy

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  • (PMID = 11368285.001).
  • [ISSN] 0012-6667
  • [Journal-full-title] Drugs
  • [ISO-abbreviation] Drugs
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] New Zealand
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 156
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