Abstract:
Hairy cell leukemia is a B-cell lymphoproliferative syndrome. This rare disease occurs in only 2% of all leukemia cases and is seen most often in older men. Malignant cell clones are usually B-lymphocytes. The characteristic cytoplasmic projections are responsible for the name of the disease.
Clinical picture:
The typical symptoms of pancytopenia include fatigue (anemia), recurrent infections (neutropenia) and bleeding (thrombocytopenia). Splenomegaly is almost always present without enlargement of the lymph nodes. Hepatomegaly also occurs in approximately 50% of the cases.
Hematology:
Pancytopenia exists where the neutropenia is most severe. Monocytopenia also occurs. A variant with increased white blood cell counts exists (presented case). Hairy cells are characteristic in the peripheral blood. These cells show a positive reaction to the tartrate-resistant acid phosphatase staining (lower image). Bone marrow aspiration often yields no material, since myelofibrosis often exists.