Splenic marginal zone lymphoma: a review of the clinical presentation, pathology, molecular biology, and management

Larissa Sena Teixeira Mendes,1 Ming-Qing Du,2 Estella Matutes,3 Andrew Wotherspoon11Histopathology Department, Royal Marsden Hospital, London, UK; 2Molecular Malignancy Laboratory and Department of Histopathology, University Hospital NHS Foundation Trust/Division of Molecular Histopathology, Depart...

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Autores principales: Teixeira Mendes LS, Du MQ, Matutes E, Wotherspoon A
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Publicado: Dove Medical Press 2014
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spelling oai:doaj.org-article:934921f055d04fdcabd27e7ea0acb0fc2021-12-02T03:33:01ZSplenic marginal zone lymphoma: a review of the clinical presentation, pathology, molecular biology, and management1179-9889https://doaj.org/article/934921f055d04fdcabd27e7ea0acb0fc2014-07-01T00:00:00Zhttp://www.dovepress.com/splenic-marginal-zone-lymphoma-a-review-of-the-clinical-presentation-p-peer-reviewed-article-BLCTThttps://doaj.org/toc/1179-9889 Larissa Sena Teixeira Mendes,1 Ming-Qing Du,2 Estella Matutes,3 Andrew Wotherspoon11Histopathology Department, Royal Marsden Hospital, London, UK; 2Molecular Malignancy Laboratory and Department of Histopathology, University Hospital NHS Foundation Trust/Division of Molecular Histopathology, Department of Pathology, University of Cambridge, Cambridge, UK; 3Hematopathology Unit, Hospital Clinic, Barcelona University, Barcelona, Spain Abstract: Splenic marginal zone lymphoma is a distinct low grade B-cell lymphoma primarily occurring in the spleen and separate from nodal marginal zone lymphoma and extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue. It is characterized by a relative indolent course, splenomegaly, moderate lymphocytosis, and an intrasinusoidal pattern of involvement, especially in the bone marrow. It is postulated that the neoplastic clone originates from persistent antigenic stimulation of marginal zone B-cells. Molecular and cytogenetic studies have failed to show specific alterations. There is no standard criterion to initiate treatment, which may include a watch and wait policy, splenectomy, or chemo/immunotherapy. This review highlights the main features of this entity, reassessing the guidelines for diagnosis, prognostic factors, staging, and management published by the SMZL Working Group (2008). Keywords: splenectomy, villous lymphocytes, guidelinesTeixeira Mendes LSDu MQMatutes EWotherspoon ADove Medical PressarticleDiseases of the blood and blood-forming organsRC633-647.5ENBlood and Lymphatic Cancer: Targets and Therapy, Vol 2014, Iss default, Pp 29-38 (2014)
institution DOAJ
collection DOAJ
language EN
topic Diseases of the blood and blood-forming organs
RC633-647.5
spellingShingle Diseases of the blood and blood-forming organs
RC633-647.5
Teixeira Mendes LS
Du MQ
Matutes E
Wotherspoon A
Splenic marginal zone lymphoma: a review of the clinical presentation, pathology, molecular biology, and management
description Larissa Sena Teixeira Mendes,1 Ming-Qing Du,2 Estella Matutes,3 Andrew Wotherspoon11Histopathology Department, Royal Marsden Hospital, London, UK; 2Molecular Malignancy Laboratory and Department of Histopathology, University Hospital NHS Foundation Trust/Division of Molecular Histopathology, Department of Pathology, University of Cambridge, Cambridge, UK; 3Hematopathology Unit, Hospital Clinic, Barcelona University, Barcelona, Spain Abstract: Splenic marginal zone lymphoma is a distinct low grade B-cell lymphoma primarily occurring in the spleen and separate from nodal marginal zone lymphoma and extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue. It is characterized by a relative indolent course, splenomegaly, moderate lymphocytosis, and an intrasinusoidal pattern of involvement, especially in the bone marrow. It is postulated that the neoplastic clone originates from persistent antigenic stimulation of marginal zone B-cells. Molecular and cytogenetic studies have failed to show specific alterations. There is no standard criterion to initiate treatment, which may include a watch and wait policy, splenectomy, or chemo/immunotherapy. This review highlights the main features of this entity, reassessing the guidelines for diagnosis, prognostic factors, staging, and management published by the SMZL Working Group (2008). Keywords: splenectomy, villous lymphocytes, guidelines
format article
author Teixeira Mendes LS
Du MQ
Matutes E
Wotherspoon A
author_facet Teixeira Mendes LS
Du MQ
Matutes E
Wotherspoon A
author_sort Teixeira Mendes LS
title Splenic marginal zone lymphoma: a review of the clinical presentation, pathology, molecular biology, and management
title_short Splenic marginal zone lymphoma: a review of the clinical presentation, pathology, molecular biology, and management
title_full Splenic marginal zone lymphoma: a review of the clinical presentation, pathology, molecular biology, and management
title_fullStr Splenic marginal zone lymphoma: a review of the clinical presentation, pathology, molecular biology, and management
title_full_unstemmed Splenic marginal zone lymphoma: a review of the clinical presentation, pathology, molecular biology, and management
title_sort splenic marginal zone lymphoma: a review of the clinical presentation, pathology, molecular biology, and management
publisher Dove Medical Press
publishDate 2014
url https://doaj.org/article/934921f055d04fdcabd27e7ea0acb0fc
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AT dumq splenicmarginalzonelymphomaareviewoftheclinicalpresentationpathologymolecularbiologyandmanagement
AT matutese splenicmarginalzonelymphomaareviewoftheclinicalpresentationpathologymolecularbiologyandmanagement
AT wotherspoona splenicmarginalzonelymphomaareviewoftheclinicalpresentationpathologymolecularbiologyandmanagement
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