Advances in the understanding of acquired haemophilia A: implications for clinical practice.
Acquired haemophilia A is an auto-immune disease caused by an inhibitory antibody to factor VIII. Patients with an acquired factor VIII inhibitor are at risk of life- and limb-threatening bleeding until the inhibitor has been eradicated. Management relies on rapid and accurate diagnosis, control of bleeding episodes, investigation for a precipitating cause and eradication of the inhibitor by immunosuppression. Patients should always be managed jointly with a specialist centre even if they present without overt bleeding. Despite an extensive literature, few controlled data are available and management guidelines are predominantly based on case reports, retrospective cohorts and expert opinion. This paper reviews the current literature on incidence, pathogenesis, diagnosis, haemostatic therapy and inhibitor eradication strategies. Potential future developments are discussed.
Collins
Peter W
PW
Arthur Bloom Haemophilia Centre, University Hospital of Wales and School of Medicine, Cardiff University, UK. peter.Collins@cardiffandvale.wales.nhs.uk
Percy
Charles L
CL
D000328:Adult; D002648:Child; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D005169:Factor VIII / Q000627:therapeutic use; D005260:Female; D006467:Hemophilia A / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy*; D006801:Humans; D007136:Immunoglobulins / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D011247:Pregnancy; D011248:Pregnancy Complications; D012008:Recurrence
D007136:Immunoglobulins; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D005169:Factor VIII; D003894:Deamino Arginine Vasopressin