Abnormalities in hemostasis in acute promyelocytic leukemia

Our understanding of the hemostatic abnormalities in acute promyelocytic leukemia (APL) has undergone remarkable changes over the past three decades. Hemorrhagic complications lead to significant morbidity and mortality in patients with APL. Up to 30% of early deaths are due to hemorrhagic complications. Our concept of the coagulation changes in APL have evolved from a simple belief that the bleeding was due to disseminated intravascular coagulation (DIC) from underlying infection, to a knowledge that the APL cells themselves are responsible for the bleeding. We have observed that apoptotic APL cells have increased generation of thrombin and therefore contribute to the hypercoagulability and DIC. In addition, excessive fibrinolysis has been recognized to play an important role. The advent of a new therapeutic approach with all-trans-retinoic acid has improved overall survival and has greatly shortened the hemorrhagic phase of the disease, but only to produce a new complication, the retinoic acid syndrome. Its pathogenesis and management still remain to be the challenge for the present. Copyright (C) 2001 John Wiley Sons, Ltd.