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Hairy-cell leukemia: A disease in which there are cancer cells in the blood and bone marrow called hairy cells because they look "hairy" when examined under a microscope. The hairy cells are malignant white blood cells of the B-cell type. Hairy-cell leukemia accounts for 2% of all cases of leukemia.

When hairy-cell leukemia develops, the leukemic cells may collect in the spleen, and the spleen may become enlarged (splenomegaly). There also may be too few normal blood cells of all types (pancytopenia) because the leukemic cells invade the bone marrow and the marrow cannot produce enough normal blood cells. The deficit of different types of normal blood cells can lead to anemia, easy bleeding, and a tendency to infection.

Diagnostic tests include blood tests to count the number of each of the different types of blood cells and a bone marrow biopsy. During this test, a needle is inserted into a bone and a small amount of bone marrow is taken out and looked at under the microscope.

Splenectomy (surgery to remove the spleen) provides palliation (some help but not a cure).

Treatment with drugs, principally interferon alfa and purine analogues (such as cladribine and pentostatin), permits the survival of the majority of patients 8 years following their initial diagnosis. For the 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.

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