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Citation:
Weerakkody Y, Bell D, Iqbal S, Hairy cell leukaemia. Reference article, Radiopaedia.org (Accessed on 03 Jul 2024) https://doi.org/10.53347/rID-90960
Hairy cell leukaemia 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 recognised 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 tumour burden and bone marrow infiltration.
Subtypes
Two main subtypes have been described
Treatment and prognosis
The combination of cladribine and pentostatin, either single or combined, with an anti-CD20 monoclonal antibody achieved stable results 7.
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6. Peterson C, Kaplan PA, Lorenzen KM. Case report 410: Hairy cell leukemia infiltrating the femora. (1987) Skeletal radiology. 16 (1): 82-6. doi:10.1007/BF00349936 - Pubmed
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7. Grever MR, Abdel-Wahab O, Andritsos LA, Banerji V, Barrientos J, Blachly JS, Call TG, Catovsky D, Dearden C, Demeter J, Else M, Forconi F, Gozzetti A, Ho AD, Johnston JB, Jones J, Juliusson G, Kraut E, Kreitman RJ, Larratt L, Lauria F, Lozanski G, Montserrat E, Parikh SA, Park JH, Polliack A, Quest GR, Rai KR, Ravandi F, Robak T, Saven A, Seymour JF, Tadmor T, Tallman MS, Tam C, Tiacci E, Troussard X, Zent CS, Zenz T, Zinzani PL, Falini B. Consensus guidelines for the diagnosis and management of patients with classic hairy cell leukemia. (2017) Blood. 129 (5): 553-560. doi:10.1182/blood-2016-01-689422 - Pubmed
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