Treatment of life-threatening hemorrhage due to acquired factor VIII inhibitor.
An otherwise healthy elderly man developed massive, life-threatening, sublingual bleeding associated with an idiopathic factor VIII inhibitor. The patient was treated wtih cyclophosphamide, steroids, factor VIII concentrates, and repeated plasmapheresis (including three times with NCI-IBM blood-cell separator). Rapid clinical and laboratory improvement occurred, with complete disappearance of the inhibitor. The patient has remained well, without evidence of an inhibitor, for 8 mo. The possible role of each of the therapeutic measures in the disappearance of the inhibitor and the possible pathogenetic mechanism of this disorder are discussed. A high mortality rate and a striking incidence of sublingual hematoma have been observed in cases in the literature.
D000368:Aged; D000906:Antibodies / Q000032:analysis; D003520:Cyclophosphamide / Q000627:therapeutic use; D005169:Factor VIII / Q000037:antagonists & inhibitors* / Q000627:therapeutic use; D006406:Hematoma / Q000188:drug therapy / Q000209:etiology*; D006801:Humans; D008297:Male; D006472:Oral Hemorrhage / Q000188:drug therapy / Q000209:etiology*; D010956:Plasmapheresis; D011241:Prednisone / Q000627:therapeutic use; D013925:Thromboplastin / Q000032:analysis
D000906:Antibodies; D003520:Cyclophosphamide; D005169:Factor VIII; D013925:Thromboplastin; D011241:Prednisone