Hairy-Cell Leukaemia

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Hairy-cell leukaemia (HCL) is an established clinicopathological entity typified by a chronic picture of substantial splenomegaly, less frequent hepatomegaly, and only inconspicuous lymphadenopathy. Central to the diagnosis is the pathognomonic hairy cell (HC), a dis­ tinctive mononuclear cell that circulates in varying numbers and infiltrates a variety of organs, including the bone marrow and spleen. Peripheral pancytopenia is often present, and circulating HCs may be infrequent. HCs are invariably present in the bone marrow, but, per­ haps because of the extensive fibrosis characteristic of the disease, the marrow is frequently difficult to aspirate, and confirmation of the diagnosis may require histopathological ex­ amination of biopsy material. The incidence of the disease has been given as 2% ofleukaemias [29, 319] or as approximately 1 % of identifiable lymphomas [230]. Our own experience of collecting cases in the United Kingdom leads us to expect that at any given time one patient with HCL will be attending a clinic serving some 150, 000 people.