Hairy cell leukemia
Chapter: 20b
Hairy cell leukemia is a low grade lymphoproliferative disorder of B-cell origin which primarily involves the spleen, bone marrow and peripheral blood. It is an uncommon disorder. The mean age is 50-60 years old, with a male predominance. Patients present with pancytopenia and splenomegaly, and usually have a diffuse infiltration of leukemic cells in the bone marrow and spleen. Diagnosis is based on the finding of "hairy" cells, due to cytoplasmic projections on the cell surface, and demonstration of characteristic tartrate-resistant acid phosphatase (TRAP) by cytochemistry. The recent use of 2-deoxycoformycin ( "2-CDA") has greatly improved the survival in these patients.