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

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, moderat...

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Published inBlood and lymphatic cancer Vol. 4; no. default; pp. 29 - 38
Main Authors Mendes, Larissa Sena Teixeira, Du, Ming-Qing, Matutes, Estella, Wotherspoon, Andrew
Format Journal Article
LanguageEnglish
Published Macclesfield Dove Medical Press Limited 01.01.2014
Taylor & Francis Ltd
Dove Medical Press
Subjects
Anemia
B cells
Bone marrow
Cancer therapies
Chemotherapy
Cytoplasm
Dehydrogenases
Diagnosis
Genetic aspects
Hepatitis
Histopathology
Lymphocytes
Lymphoma
Lymphomas
Pathology
Physiological aspects
Risk factors
Spleen
Spain
United Kingdom > UK
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Summary: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
ISSN:1179-9889
1179-9889
DOI:10.2147/BLCTT.S49373