A chronic lymphocytic leukemia consisting of mantle cells may follow a slow, indolent course. Aggressive management may do more harm than good for these patients.
Features:
(1) chronic lymphocytosis with cells marking with CD5, CD19 and CD20
(2) cytogenetics shows a simple karyotype with t(11;14)(q13;q32), resulting in an overproduction of cyclin D1
(3) usually a kappa light chain restriction (mantle cell lymphoma often shows lambda light chain restriction)
(4) little or no lymphadenopathy, no splenomegaly and no overt gastrointestinal involvement
(5) bone marrow shows a low level interstitial infiltrate of single lymphocytes or small aggregates
(6) clinically asymptomatic
Additional features:
(1) normal to minimally elevated serum LDH
(2) lack of SOX11 expression (a nuclear transcription factor)
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