B-cell prolymphocytic leukaemia
§ Prolymphocytes >55% of lymphocytes in PB § Very rare § Occurs de novo or transformation of CLL
Clinical features § Elderly § Marked splenomegaly without peripheral lymphadenopathy § Rapidly rising lymphocyte count (usually >100) § Serum M-component in some
Blood/ Bone marrow § Double the size of small lymphocytes with prominent nucleoli § Diffuse intertrabecular infiltration § Pseudofollicles not seen
Immunophenotype § Strong expression of SIg M +/-D § B cell antigens § CD5 + in 1/3 § CD23 negative § FMC7+
Cytogenetics § T(11;14) in 20% § Difficult to tell apart from blastoid variant of Mantle Cell § P53 mutations have been reported
Treatment § Responds to fludaribine and cladribine but less well than CLL does § CHOP § Splenic irradiation / splenectomy for symptomatic enlargement or cytopenias
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Haemato-oncology > Mature B Cell neoplasms >