[Elderly non-hemophiliac patient with factor VIII inhibitor presenting various type hemorrhages]

A 81-year-old man who had been healthy without any history of abnormal bleeding, developed ecchymosis and hematuria in November, 1992 and was hospitalized in December, 1992. On admission, he developed widespread ecchymosis in his trunk and extremities, and subsequently ecchymosis of his cheek and neck, and also oral and pharyngeal hematoma. The laboratory data were as follows: whole blood clotting time, > 20 minutes; activated partial thromboplastin time (APTT), 108.6 seconds; Factor VIII activity, 4%. The level of Factor VIII inhibitor was high, 65.0 Bethesda Unit/ml. This inhibitor was a IgG type immunoglobulin, which had both kappa and lambda light chain. His serological and blood biochemical data of the blood were normal, and tests for autoantibodies were negative. The patient was treated with plasma exchange therapy, Prednisolone (PSL), Cyclophosphamide and Factor VIII concentrate. The hemorrhagic symptoms were improved, the inhibitor disappeared and the activity of Factor VIII returned to normal after one month. Follow-up was continued in the outpatient clinic for 5 months. After the dose of PSL was decreased, he developed bloody sputum and hematuria, and was readmitted in August, 1994. Factor VIII activity was 21% and the titer of Factor VIII inhibitor was 3.0 BU/ml. The hemorrhagic symptoms disappeared soon after increasing the dose of PSL, and the Factor VIII activity was normalized and the inhibitor could not be detected. These treatments appeared to offer effective control on severe hemorrhage in a patient with Factor VIII inhibitor.