Coagulopathy of acute promyelocytic leukemia

Life-threatening bleeding is frequent in acute leukemias, particularly in a cute promyelocytic leukemia (APL), a distinct subtype of acute myelogenous leukemia, characterized by the balanced reciprocal translocation between ch romosomes 15 and 17. Laboratory assessments show profound hemostatic imbala nce compatible with the clinical picture of disseminated intravascular coag ulation. Activation of the coagulation system, hyperfibrinolysis and nonspe cific proteases activity can be observed in this condition. An important pa thogenetic role is attributed to the leukemic cell properties for activatin g hemostatic mechanisms. This review will summarize what is currently known about the coagulopathy of APL, the principal pathogenetic mechanisms, and the therapeutic tools for the management of this complication. Special atte ntion will be devoted to the new therapy with all-trans retinoic acid, whic h has completely changed the natural history of APL and APL-related coagulo pathy. Copyright (C) 2001 S. Karger AG, Basel.