Saturday, 12 November 2011

Hairy cell leukemia

- rare leukemia, 2% of lymphoid leukemia
- male:female = 4:1
- hairy cells are larger than lymphocytes, their nuclei show a homogenous loose chromatic pattern without a visible nucleolus and cells have abundant cytoplasm with broad base projection or villi, nuclear outline is often kidney shape
- presents with pancytopenia and massive splenomegaly
- associated with monocytopenia (typical finding)
- skin vasculitis (erythema nodosum & cutaneous nodules) in one third of patients
- bone marrow trephine biopsy: characteristic clear zone between cell (fried egg appearance)
- increased bone marrow reticulin often results in dry tap marrow aspirate despite marrow hypercellularity
- abnormal cells are strongly positive for tartrate resistant acid phosphatase strain (TRAP)
- respond dramatically to purine analogues (cladribine and pentostatin), with survival rate at 5 years
of 95-95%

1 comment:


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