Hairy cell leukemia: A form of chronic leukemia in which malignant B-lymphocytes (a type of white blood cell ) are seen in the bone marrow , spleen , and peripheral blood and when viewed under the microscope, these cells appear to be covered with tiny hair-like projections. Hairy cell leukemia represents 2% of all leukemia.

The characteristic features of the disease include marked enlargement of the spleen ( splenomegaly ), low blood cell counts ( pancytopenia ), a relatively small number of circulating tumor cells with a hairy appearance, and infiltration of the spleen, liver, and bone marrow by the leukemic hairy cells.

Annexin A1 (ANXA1) is a gene that is unregulated in hairy cell leukemia. ANXA1 protein expression is specific to hairy cell leukemia. Detection of ANXA1 (by immunocytochemical means) provides a simple, highly sensitive and specific assay for the diagnosis of hairy cell leukemia.

Splenectomy (surgical removal of the spleen) provides palliation (some help but not a cure). Treatment is usually with drugs, principally interferon alfa and purine analogues such as cladribine and pentostatin. For resistant cases, a promising immunotoxin has been developed that targets CD22, a molecule expressed exclusively on the surface of B-cells, including virtually all hairy cells.

Hairy cell leukemia was first described in 1958 and was misleadingly called leukemic reticuloendotheliosis.

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