Case 69 – part 2 – summary

This patient has a medical emergency. She has pancytopenia with evidence of bleeding. There are abnormal promyelocytes and faggot cells (below) which are indicative of acute promyelocytic leukaemia.

case-69-part-2b

Acute promyelocytic leukaemia is a subtype of acute myeloid leukaemia characterised by t(15;17) in the leukaemic clone resulting in the PML-RARA fusion protein. The survival of APL is excellent but the biggest risk is the initial period of diagnosis where coagulopathy can result in catastrophic bleeding. For this reason therapy must be instituted immediately with all-trans retinoic acid and blood products (platelets, fresh frozen plasma, cryoprecipitate) to correct the coagulopathy. ATRA is combined with idrarubicin (AIDA regime) or arsenic trioxide with very high cure rates.

 

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