DICによる後天性無フィブリノゲン血症に対し大量フィブリノゲン投与にて救命されたAPLの1例

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  • Life-saving Administration of Fibrinogen to a Case of APL with Acquired Afibrinogenemia due to Disseminated Intravascular Coagulation

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A female patient with acute promyelocytic leukemia and severe bleeding tendency with acquired afibrinogenemia is described. On admission, her plasma fibrinogen was markedly decreased beyond the range of measurement (<10mg/dl). Although disseminated intravascular coagulation existed undoubtedly, the low circulating plasminogen and α 2-plasmin inhibitor, and the presence of plasmin-α 2 plasmin inhibitor complex suggested that the excessive fibrinolysis probably resulted from the liberation of plasminogen activator from the leukemic promyelocytes. To prevent lethal hemorrhage undergoing induction chemotherapy, we administered fibrinogen (5g/day) added to heparin, FUT-175, FFP and platelet transfusion. With these treatments, hypercoagulopathy and increased fibrinolytic state were well controlled to the extent that she did not have overt bleeding tendencies. Although the fibrinogen preparations always carry the risk of causing viral hepatitis, we believe the value of intensive supportive therapy including fibrinogen preparations to prevent the early death for same pathogenesis of severe hypofibrinogenemia.

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