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The clinical course of a patient presenting with thrombocytopenia (86 103/L) and signs of intravascular coagulation (prothrombin time, 45%; partial thromboplastin time, 49 s; fibrinogen, 40 mg/dl; antithrombin III, 85%; factor X, 73%; plasminogen, 42%) due to a giant hemangioma of the liver (Kasabach-Merritt syndrome) is reported. Treatment with i.v. heparin, fibrinogen, and fresh-frozen plasma led to significant elevation of fibrinogen (156 mg/dl) and antithrombin III (102%) without changing the decreased activities of the procoagulant factors. Similarly, the signs of hyperfibrinolysis persisted (fibrinogen degradation products, 50 μg/dl; plasminogen, 43%; alpha-2 antiplasmin, 74%). The hemangioma was completely removed by excision of the left lobe of the liver. Subsequently, all coagulation parameters returned to normal, indicating a complete reversibility of the coagulation disorder.