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General Medicine · Haematology: hairy cell leukaemia
Sample itemA 55-year-old man has fatigue, recurrent infections, a palpable spleen 8 cm below the costal margin and no lymphadenopathy. Counts show pancytopenia with monocytopenia, and bone marrow aspiration gives a dry tap. Flow cytometry shows CD11c, CD25 and CD103 positive B cells with a BRAF V600E mutation. What is the treatment of choice?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
A. Cladribine
Hairy cell leukaemia is an indolent B-cell neoplasm with splenomegaly, pancytopenia, a dry tap from marrow fibrosis, and cells that are tartrate-resistant acid phosphatase positive with a BRAF V600E mutation. A single course of cladribine, a purine analogue, induces long remissions in most patients. Splenectomy and interferon are historical options.
Option by option
A. Cladribine (correct)
Correct. A purine analogue achieves high complete response rates in hairy cell leukaemia.
B. All-trans retinoic acid with arsenic trioxide
ATRA with arsenic trioxide treats acute promyelocytic leukaemia with t(15;17).
C. Cyclophosphamide, doxorubicin, vincristine and prednisolone (CHOP) every 3 weeks
CHOP is used for aggressive lymphomas and is not standard for this indolent leukaemia.
D. Imatinib
Imatinib inhibits BCR-ABL in chronic myeloid leukaemia and has no activity here.
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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