Review Article
Year: 2020 | Month: October-December | Volume: 5 | Issue: 4 | Pages: 369-375
Acquired Hemophilia: An Overview
Pandeep Kaur1, Shatakshi Jindal2, Gita Negi3, Yatendra Mohan1, Jyotsna Bhateja1
Senior Resident1, Junior Resident2, Additional Professor and Head3,
All India Institute of Medical Sciences, Rishikesh.
Corresponding Author:Shatakshi Jindal
ABSTRACT
Acquired hemophilia A(AHA) is a rare disorder, having an incidence of approximately one per million per year with a one in five mortality rate. It is due to autoantibodies against coagulation factor VIII which neutralizes the procoagulant activity and results in severe and life-threatening bleeding. It is seen in patients with no prior history of hemophilia A. It may be associated with pregnancy, autoimmune diseases, malignancy, infections or medication. It occurs mostly in elderly. Approximately 50% of the patients have no underlying pathological conditions. Clinical manifestations include spontaneous hemorrhages in the skin, muscles or soft tissues or excessive bleeding during surgery. Hemarthrosis, hallmark of congenital severe hemophilia A is seldom seen. The diagnosis is based upon reduced FVIII levels and isolated prolongation of activated partial thromboplastintime (aPTT) which does not normalize after addition of normal plasma. The treatment involves removal of antibodies and maintaining effective hemostasis during bleeding episodes. Treatment options for removal of antibodies are immunoadsorption, immunosuppression or immune tolerance induction (ITI). Treatment of acute bleeding episodes includes use of bypassing agents like recombinant activated factor VII and activated prothrombincomplex concentrate in patients with high titer of inhibitors or antifibrinolytics, 1-deamino-8-D-arginine vasopressin (DDAVP) or FVIII concentrates in patients having low titer of inhibitors. Rituximab, an anti-CD20 monoclonal antibody can be used alone or in combination with immunosuppressive therapy in patients not responding to standard immunosuppressors.
Keywords: Acquired hemophilia A(AHA), hemophilia A.