Hairy cell leukemia
Updates to Article Attributes
Hairy cell leukaemia is a rare and indolent form of small mature B-cell leukaemias.
Epidemiology
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 recognised male predilection of around 4:1 with a median age of around 55 years.
Pathology
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 tumour burden and bone marrow infiltration
Subtypes
Two main subtypes have been described
- classical hairy cell leukaemia: 90%
- hairy cell leukaemia variant (HCL-V): 10%
Treatment and prognosis
The combination of the cladribine and pentostatin either single or in hybrid with an anti-CD20 monoclonal antibody achieved stable results 7.
See also
-<a title="hairy cell leukaemia variant" href="/articles/hairy-cell-leukaemia-variant">h</a><a href="/articles/airy-cell-leukaemia-variant">airy cell leukaemia variant </a>(HCL-V): 10%</li>- +<a href="/articles/hairy-cell-leukaemia-variant">h</a><a href="/articles/airy-cell-leukaemia-variant">airy cell leukaemia variant </a>(HCL-V): 10%</li>
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