Malignancy is associated with a "hypercoagulable" state and a high risk for thrombohemorrhagic complications. Clinical complications may range from localized
thrombosis to
bleeding of varying degrees of severity because of
disseminated intravascular coagulation (
DIC). Life-threatening
bleeding is frequent in acute
leukemias, particularly in
acute promyelocytic leukemia (APL). Laboratory assessments show profound
hemostatic imbalance in this condition, with activation of coagulation, fibrinolysis, and nonspecific proteolysis systems. An important pathogenetic role is attributed to the leukemic cell properties interfering with the
hemostatic mechanisms. However,
chemotherapy and intercurrent
infections also contribute to the
bleeding risk in the patient with
leukemia. In this article, we will attempt to describe what is currently known about the coagulopathy of acute
leukemia, summarize the various aspects of the
hemostatic abnormalities underlying this disorder, and revise the principal pathogenetic mechanisms. We will also try to provide information on the current therapeutic tools and recommendations for the management of life-threatening
bleeding in this disease. Finally, a special focus will be devoted to the management of this complication in the era of
all-trans retinoic acid (ATRA), a drug now indispensable in curing APL that has completely changed the natural history of APL and its coagulation/
bleeding syndrome.