Splenic marginal zone lymphoma

Blood Rev. 2005 Jan;19(1):39-51. doi: 10.1016/j.blre.2004.03.002.


Splenic marginal zone lymphoma (SMZL) is an indolent B cell malignancy usually involving spleen, bone marrow and blood. The disease presents as an incidental finding or with symptoms of splenic enlargement or anaemia. Diagnosis is based on a combination of lymphocyte morphology, immunophenotype and marrow and /or splenic histology. There is no genetic abnormality specific for SMZL, but deletions of chromosome 7q are the commonest abnormality and are found in 30-50% of cases. SMZL cells may have either mutated or unmutated immunoglobulin variable region genes and probably arise from different subsets of splenic marginal zone B cells. Prognostic factors are poorly defined and only loss or mutation of the p53 gene is consistently associated with a poor outcome. Therapeutic options include splenectomy, splenic irradiation, alkylating agents, purine analogues or anti CD20 antibody. The median survival is 10-13 years and most disease-related deaths are associated with transformation to diffuse large cell lymphoma.

Publication types

  • Review

MeSH terms

  • DNA Mutational Analysis
  • Gene Expression Profiling
  • Humans
  • Immunophenotyping
  • Lymphoma, B-Cell / diagnosis
  • Lymphoma, B-Cell / genetics
  • Lymphoma, B-Cell / therapy
  • Mutation
  • Splenic Neoplasms* / diagnosis
  • Splenic Neoplasms* / genetics
  • Splenic Neoplasms* / therapy