Hairy cell leukemia

Last revised by Daniel J Bell on 6 Jul 2021

Hairy cell leukemia is a rare and indolent form of small mature B-cell leukaemias.

Its annual incidence is estimated at around 0.3 cases per 100 000, and the disease comprises 2-3% of all leukaemias. There is a recognized male predilection of around 4:1 with a median age of around 55 years.

It can present with splenomegaly, vulnerability to recurrent infections, pancytopenia and bone marrow involvement with fibrosis.

Skeletal lesions uncommonly occur (~3% of cases) and when they do are commonly lytic with the most common sites of involvement being the femoral head and neck 5. Skeletal involvement is typically associated with high tumor burden and bone marrow infiltration.

Two main subtypes have been described

The combination of cladribine and pentostatin, either single or combined, with an anti-CD20 monoclonal antibody achieved stable results 7.

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