Splenic marginal zone lymphoma.
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.
Oscier
David
D
Department of Haematology, Royal Bournemouth Hospital, Castle Lane East, Bournemouth BH7 7DW, UK. david.oscier@lineone.net
Owen
Roger
R
Johnson
Stephen
S
D004252:DNA Mutational Analysis; D020869:Gene Expression Profiling; D006801:Humans; D016130:Immunophenotyping; D016393:Lymphoma, B-Cell / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D009154:Mutation; D013160:Splenic Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000628:therapy