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HYPOFIBRINOGENEMIA DUE TO INCREASED FIBRINOLYSIS IN TWO PATIENTS WITH ACUTE PROMYELOCYTIC LEUKEMIA
Authors:T K CHAN  G T C CHAN  VIVIAN CHAN
Institution:Professor in Medicine, University Department of Medicine, Queen Mary Hospital, Hong Kong;Clinical Pathologist, Department of Pathology, University Department of Medicine, Queen Mary Hospital, Hong Kong;Reader in Medicine, University Department of Medicine, Queen Mary Hospital, Hong Kong
Abstract:Two patients with acute promyelocytic leukemia and severe bleeding associated with hypofibrinogenemia were studied. The markedly shortened whole blood clot lysis time and dilute clot lysis time suggested that the defect was an increase in fibrinolysis. Although disseminated intravascular coagulation could not be totally excluded as an alternative mechanism, excessive fibrinolysis was confirmed as the pathogenic cause by the prompt response to the administration of tranexamic acid. The low circulating plasminogen, α2 plasmin inhibitor level and the presence of α2 plasmin inhibitor-protease complex in both patients suggested that the increased fibrinolysis probably resulted from the liberation of plasminogen activator from the promyelocyte.
Keywords:Hypofibrinogenemia  fibrinolysis  tranexamic acid  acute promyelocytic leukemia
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