Giant hemangioma of the liver (Kasabach-Merritt syndrome): successful suppression of intravascular coagulation permitting surgical removal.
The clinical course of a patient presenting with thrombocytopenia (86 X 10(3)/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 micrograms/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.
The Second Department of Internal Medicine, University of Vienna, Austria.
H H
HH
Watzke
W
W
Linkesch
U
U
Hay
D000328:Adult; D004211:Disseminated Intravascular Coagulation / Q000209:etiology* / Q000628:therapy; D005260:Female; D005340:Fibrinogen / Q000627:therapeutic use; D006391:Hemangioma / Q000150:complications* / Q000601:surgery; D006493:Heparin / Q000627:therapeutic use; D006801:Humans; D008113:Liver Neoplasms / Q000150:complications* / Q000601:surgery; D010949:Plasma; D013577:Syndrome
D005340:Fibrinogen; D006493:Heparin