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Pediatrics · Systemic paediatrics: acute promyelocytic leukaemia
Sample itemA 6-year-old has bleeding gums and purpura. The blood count shows pancytopenia with abnormal promyelocytes containing multiple bundles of Auer rods. PT and aPTT are prolonged and fibrinogen is low. Which drug should be started immediately with supportive care?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
D. All-trans retinoic acid
The picture is acute promyelocytic leukaemia with DIC, with t(15;17) producing PML-RARA. All-trans retinoic acid induces differentiation, reduces the coagulopathy and is started on suspicion, usually with arsenic trioxide or chemotherapy.
Option by option
A. Imatinib mesylate
Imatinib targets BCR-ABL in chronic myeloid leukaemia and Philadelphia-positive ALL.
B. Intrathecal methotrexate
Intrathecal methotrexate is for central nervous system prophylaxis in lymphoblastic leukaemia.
C. Rituximab
Rituximab targets CD20 on B cells and has no role in this myeloid leukaemia.
D. All-trans retinoic acid (correct)
Correct. All-trans retinoic acid induces differentiation and corrects the coagulopathy of this leukaemia.
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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