Long-term high dose factor VIII treatment of 3 haemophiliacs with factor VIII inhibitor.
3 patients with haemophilia A and inhibitor against Factor VIII were developing progressive haemophiliac arthropathy due to the non-feasibility of prophylactic treatment. In order to suppress inhibitor formation, long-term treatment with high-dose Factor VIII (100 units per kg body weight twice daily) was initiated. Prothrombin complex concentrate was given only for bleeding episodes. Though all 3 patients were high responders, they presented different treatment courses. 2 became low responders after 4 and 11 months' treatment, respectively. 1 patient had no demonstrable inhibitor after start of treatment. In all 3 patients, prophylactic treatment was established, in 1 case still with increased doses compared to non-inhibitor patients. The high-dose Factor VIII treatment makes in possible to provide prophylactic treatment for the high-responder inhibitor patients. However, the extremely high costs represent a serious obstacle to this treatment.
D000293:Adolescent; D000906:Antibodies / Q000032:analysis*; D000941:Antigens / Q000032:analysis; D002648:Child; D005167:Factor VII / Q000032:analysis / Q000276:immunology; D005169:Factor VIII / Q000378:metabolism / Q000627:therapeutic use*; D005500:Follow-Up Studies; D006467:Hemophilia A / Q000097:blood / Q000188:drug therapy*; D006801:Humans; D008297:Male; D011516:Prothrombin / Q000627:therapeutic use; D014841:von Willebrand Factor / Q000032:analysis
D000906:Antibodies; D000941:Antigens; C031500:factor VII related antigen; D014841:von Willebrand Factor; D005167:Factor VII; D011516:Prothrombin; D005169:Factor VIII