Management of acquired haemophilia A--more questions than answers.
This paper discusses selected reports on the management of acquired haemophilia A, a rare bleeding disorder characterized by the development of an autoantibody directed against plasma coagulation factor VIII (FVIII) (Morrison et al., Blood 81:1513-1520, 1993). The current literature consists of reports of cohorts of patients from referral centres and retrospective surveys of referral centres (Green and Lechner, Thromb Haemost 45:200-203, 1981). This suggests that the current literature may be biased both by referral practice to tertiary centres and reporting bias of these centres and may not be representative of the full spectrum of the disease. The published studies describe various immunosuppressive regimens to eradicate factor VIII inhibitors but usually lack control patients. Only one report describes a randomized, controlled study. Studies that address treatment of bleeding episodes give data on safety and efficacy for individual products but no comparative studies are available.
Arthur Bloom Haemophilia Centre, University Hospital of Wales, Heath Park, Cardiff, UK. collinspw@cardiff.ac.uk
Peter W
PW
Collins
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000818:Animals; D001323:Autoantibodies / Q000276:immunology; D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology / Q000276:immunology; D015982:Bias; D001779:Blood Coagulation Factors / Q000627:therapeutic use; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D005169:Factor VIII / Q000276:immunology / Q000627:therapeutic use; D005260:Female; D006467:Hemophilia A / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology / Q000276:immunology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007163:Immunosorbent Techniques; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D006473:Postpartum Hemorrhage / Q000188:drug therapy / Q000209:etiology; D011247:Pregnancy; D012189:Retrospective Studies; D013552:Swine
D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D001779:Blood Coagulation Factors; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; C025667:prothrombin complex concentrates; D005169:Factor VIII; D003894:Deamino Arginine Vasopressin