splenic marginal zone B-cell lymphoma
Splenic marginal zone lymphoma (SMZL) is an indolent lymphoproliferative disease accounting for approximately 1% of all lymphomas.
Clinical synopsis
marked splenomegaly
circulating atypical ’villous lymphocytes’
Microscopical synopsis
nodular infiltrate based on white pulp and red pulp
white pulp:
- biphasic morphology: an inner zone of small lymphocytes and a peripheral (marginal) zone of larger lymphoid cells
splenic lymph nodes and bone marrow: presence of a vaguely nodular infiltrate of similar nature.
Immunophenotype
Tumor cells has a mature B-cell phenotype
- IgM+
- IgD+
- CD5-
- CD23-
- CD43-
- CD10-
- Bcl-6- (BCL6-)
- cyclin-D1-
Molecular biology
Analysis of immunoglobulin heavy-chain gene variable regions suggest that some cases of SMZL arise form postfollicular B cells but others from naive B cells.
abnormalities of a number of chromosomes
- 7q31-32 LOH
Differential diagnosis
lymphoid hyperplasias
other marginal zone lymphomas,
mantle cell lymphoma
follicular lymphoma
B-CLL
See also
cutaneous marginal zone B-cell lymphoma
References
Defining the borders of splenic marginal zone lymphoma: a multiparameter study. Dufresne SD, Felgar RE, Sargent RL, Surti U, Gollin SM, McPhail ED, Cook JR, Swerdlow SH. Hum Pathol. PMID: #20004934#
Taddesse-Heath L, Pittaluga S, Sorbara L, Bussey M, Raffeld M, Jaffe ES. Marginal zone B-cell lymphoma in children and young adults. Am J Surg Pathol. 2003 Apr;27(4):522-31. PMID: #12657939#
Dogan A, Isaacson PG. Splenic marginal zone lymphoma. Semin Diagn Pathol. 2003 May;20(2):121-7. PMID: #12945935#