Acute Promyelocytic Leukemia with Disseminated Intravascular Coagulation

Abstract

Three patients with acute promyelocytic leukemia, all of whom had rapidly fatal downhill courses associated with marked bleeding tendencies, are described. Two of the three patients had hypofibrinogenemia. At autopsy, all patients had extensive hemorrhage involving internal organs, including the heart, liver, spleen, kidneys and brain. Massive hemorrhagic pulmonary edema was found in two patients. Striking leukemic involvement of the bone marrow was found, but only minimal to mild involvement of the spleen, liver, and lymph glands was evident. In addition, in all three cases autopsy disclosed evidence of disseminated intravascular clotting in small blood vessels. The significance of this finding in relation to the hypofibrinogenemia and bleeding phenomena encountered is discussed. This finding provides further support in favor of consumption (defibrination) as the cause of the bleeding diathesis in this disease.