- Epidemiology
- Most common in middle-aged men
- ♂ > ♀ (4:1)
- Pathology: mature B-cell tumor; BRAF mutations are common
- Clinical features
- Symptomatic pancytopenia
- Massive splenomegaly
- No lymphadenopathy
- B symptoms are rare.
- Diagnostics
- Usually tartrate-resistant acid phosphatase (TRAP) stain positive
- Flow cytometry (preferred over TRAP stain): CD11c marker
- CBC: Leucopenia is common but up to 20% of patients have leukocytosis.
- Peripheral blood smear: Hairy cells have irregular cytoplasmic projections that cause the characteristic “hairy” appearance.
- Bone marrow aspiration: often yields a dry tap due to bone marrow involvement with subsequent fibrosis