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Pathology · Haematology: hairy cell leukaemia
Sample itemA 55-year-old man has pancytopenia and massive splenomegaly without lymphadenopathy, and marrow aspiration gives a dry tap. Flow cytometry shows CD25 and CD103 positive B cells with cytoplasmic projections that stain tartrate-resistant acid phosphatase positive. What is the drug of choice?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
C. Cladribine
Hairy cell leukaemia is an indolent B-cell neoplasm with a BRAF V600E mutation. The cells infiltrate the marrow and red pulp of the spleen, causing pancytopenia and splenomegaly. Purine analogues, most commonly cladribine, produce long remissions and are first-line.
Option by option
A. Hydroxyurea
Hydroxyurea is a cytoreductive drug used in polycythaemia vera, sickle cell disease and CML, not first-line for this disease.
B. Imatinib
Imatinib is used for BCR-ABL positive chronic myeloid leukaemia and is not effective here.
C. Cladribine (correct)
Correct. Cladribine, a purine analogue, induces durable remission in most patients.
D. All-trans retinoic acid
All-trans retinoic acid treats acute promyelocytic leukaemia with PML-RARA.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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