905 ASSOCIATION OF A PLATELET AGGREGATION DEFECT WITH HEPATOCELLULAR CARCINOMA

Abstract

Hepatocellular carcinoma has been associated with various coagulation abnormalities, including dysfibrinogenemia and production of a biochemically-abnormal prothrombin. A 10 year old girl presented with recent onset of severe epistaxis and was found to have hepatocellular carcinoma. The bleeding time (Ivy method) was prolonged (20 min.) and factor VIII-related antigen was electrophoretically abnormal, while factor VIII-procoagulant activity protein was normal. Platelet aggregation studies demonstrated abnormal and delayed agglutination in the presence of ristocetin (0.8-1.0 mg/ml), with essentially normal agglutination with collagen, ADP, and epinephrine. A major hepatic resection (trisegmentectomy) was performed, and the tumor was completely removed. Substantial bleeding was encountered at operation despite pre-treatment with cryoprecipitate and platelet infusions, but following surgery there were no further bleeding symptoms and the bleeding time and platelet aggregation tests returned to normal. The tumor was established in long-terra culture to seek evidence for a postulated tumor-derived anticoagulant in this patient, possibly acting by interference with von Willebrand's factor. The case demonstrates that the occurrence of severe bleeding in a previously healthy child may be a manifestation of underlying hepatic malignancy.