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Antifibrinolytic therapy in the management of the kasabach merritt syndrome

1988; Wiley; Volume: 29; Issue: 1 Linguagem: Inglês

10.1002/ajh.2830290111

1096-8652

Thomas L. Ortel, James J. Onorato, Camille L. Bedrosian, Russel E. Kaufman,

Blood Coagulation and Thrombosis Mechanisms

Abstract The Kasabach Merritt syndrome consists of thrombocytopenia, microangiopathic he‐molytic anemia, and a localized consumption coagulopathy that develops within the abnormal vascular channels of a hemangioma. In general, these patients demonstrate only mild abnormalities of screening clotting tests, but they can potentially develop life‐threatening complications. We present a patient who developed a severe anemia that was refractory to erythrocyte transfusions. Treatment with epsilon‐aminocaproic acid to inhibit fibrinolysis and cryoprecipitate to replenish his deficient circulating fibrinogen interrupted the cycle of his systemic coagulopathy and enabled us to transfuse him to a normal hematocrit.