Coagulopathy in acute promyelocytic leukaemia: Can we better predict and prevent catastrophic bleeding?

Acute promyelocytic leukaemia is a distinct subset of acute myeloid leukaemia characterised by severe coagulopathy. Overall, patient prognosis is excellent, with outcomes revolutionized by the advent of all-trans retinoic acid and arsenic trioxide-based regimens. However, despite these advances, early mortality from coagulopathy and severe haemorrhage poses a major challenge, with no clear improvement seen over the past few decades. In this review, we discuss the degree of the problem, the underlying pathogenesis, and attempt to assess bleeding and mortality risk. We consider the role of leukaemia-directed therapy in the management of coagulopathy and discuss current consensus recommendations for transfusion approaches and the evidence underpinning them. We also discuss the potential future role of viscoelastic testing in this field and ongoing key research questions.