Clinical and laboratory features

• Non-specific symptoms, e.g. fatigue.

• Affects older patients, mean age at diagnosis 72 years. 182 • Moderate to massive splenomegaly.

• Lymphadenopathy rare.

• Anaemia and thrombocytopenia in 25-30% usually due to hyper-splenism. Neutropenia not marked.

• Total WBC not grossly elevated (usually <40 x 109/L; cf. PLL).

• Monocytopenia not a feature (cf. HCL)

• Cell morphology: larger than typical CLL cells, round/oval nuclei, villous cytoplasmic projections at one/both poles of the cells.

• Immunophenotype: CD19+, FMC7+, CD23- usually negative for CD5 & CD25: 20% +ve but fail to co-express CD11c and CD103 differentiating SLVL from HCL; lOtaMe p174.

• Monoclonal IgM or IgG paraprotein; free urinary light chains in 66%.

• BM aspirate may show lymphocytosis (some plasmacytoid) with typical immunophenotype; biopsy may be normal but usually shows patchy/nodular lymphoid infiltration.

• Spleen histology: characteristic with nodular infiltration involving the white pulp (cf. HCL).

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