• 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. Pasted image 20240307150238.png
    • Bone marrow aspiration: often yields a dry tap due to bone marrow involvement with subsequent fibrosis