Natural History

Hairy Cell Leukemia (HCL) is a chronic lymphoprolifer-ative disorder usually presenting with pancytopenia, recurrent systemic infections, and splenomegaly.1 The major cause of death in untreated patients is infection, usually due to an immune deficiency state related to neutropenia, monocytopenia, natural killer cell deficiency, and dendritic cell deficiency.2 3 Therapy prior to 1980 temporarily improved blood counts, but had no dramatic effect on the immune deficiency or overall survival, with patients dying as a result of their disease within 5 years of diagnosis.4 The dramatic change in survival as a result of the treatments outlined in Chapter 31 has transformed HCL from a fatal disorder to a chronic one, with some patients cured of their disease.

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