The Coagulopathy of Acute Leukemia

Abstract

Several investigations using markers specific for blood coagulation activation have demonstrated that intravascular thrombin generation accompanied by reactive hyperfibrinolysis is a key event in the coagulopathy of acute leukemia (AL). This is the main mechanism responsible for the coagulation abnormalities commonly observed in AL, especially of the promyelocytic subtype (APL). Accordingly, the changes produced on circulating substrates are described as disseminated intravascular coagulation (DIC). However, this should not imply that fibrinolytic or proteolytic activities released by leukemic celts do not contribute, in addition to clot-promoting substances, to the complex laboratory and clinical picture observed in these patients. Several studies have focussed on the clotting abnormalities found in patients with APL, in whom the rate of hemorrhagic deaths is particularly high, in order to define the best antihemorrhagic treatment for the prevention of such complications. Clinical studies, which are mostly anecdotal or retrospective, do not provide evidence to support the fact that heparin and/or antifibrinolytic treatment are associated with a lower hemorrhagic mortality. Therefore, the only recommendation that can presently be made is that of immediate inductive chemotherapy, accompanied by a generous platelet transfusion policy.