Splenic marginal zone lymphoma

Ahmet Dogan, Peter G. Isaacson

Research output: Contribution to journalArticle

36 Citations (Scopus)

Abstract

Splenic marginal zone lymphoma (SMZL) is an indolent lymphoproliferative disease accounting for approximately 1% of all lymphomas. SMZL presents with marked splenomegaly, and often accompanied by circulating atypical 'villous lymphocytes' and is also known as splenic lymphoma with villous lymphocytes. Histologically, the spleen in SMZL is characterised by a nodular infiltrate based on pre-existing white pulp but also involving the red pulp. Within the white pulp, the infiltrate has a biphasic morphology comprising an inner zone of small lymphocytes and a peripheral (marginal) zone of larger lymphoid cells. Usually the splenic lymph nodes and bone marrow are also involved by a vaguely nodular infiltrate of similar nature. Immunophenotypically, the tumor cells has a mature B-cell phenotype and frequently express IgM and IgD but typically lack CD5, CD23, CD43, CD10, Bcl-6 and cyclin D1. Analysis of immunoglobulin heavy-chain gene variable regions suggest that some cases of SMZL arise form postfollicular B cells but others from naïve B cells. Genetic studies have shown abnormalities of a number of chromosomes however 7q31-32 allelic loss appears to be characteristic. Histological differential diagnosis include a number of entities such as lymphoid hyperplasias, other marginal zone lymphomas, mantle cell lymphoma, follicular lymphoma, and B-CLL.

Original languageEnglish (US)
Pages (from-to)121-127
Number of pages7
JournalSeminars in Diagnostic Pathology
Volume20
Issue number2
DOIs
StatePublished - May 2003
Externally publishedYes

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Lymphoma
Lymphocytes
B-Lymphocytes
Immunoglobulin Heavy Chain Genes
Mantle-Cell Lymphoma
Immunoglobulin D
Follicular Lymphoma
Loss of Heterozygosity
Cyclin D1
Splenomegaly
Hyperplasia
Immunoglobulin M
Differential Diagnosis
Spleen
Chromosomes
Lymph Nodes
Bone Marrow
Phenotype
Neoplasms

Keywords

  • Marginal zone lymphoma
  • Spleen
  • Splenic lymphoma with villous lymphocytes

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Medical Laboratory Technology

Cite this

Splenic marginal zone lymphoma. / Dogan, Ahmet; Isaacson, Peter G.

In: Seminars in Diagnostic Pathology, Vol. 20, No. 2, 05.2003, p. 121-127.

Research output: Contribution to journalArticle

Dogan, Ahmet ; Isaacson, Peter G. / Splenic marginal zone lymphoma. In: Seminars in Diagnostic Pathology. 2003 ; Vol. 20, No. 2. pp. 121-127.
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