International Journal of Science and Healthcare Research Vol.5; Issue: 4; Oct.-Dec. 2020 Website: ijshr.com Review Article ISSN: 2455-7587 International Journal of Science and Healthcare Research (www.ijshr.com) 369 Vol.5; Issue: 4; October-December 2020 Acquired Hemophilia: An Overview Pandeep Kaur 1 , Shatakshi Jindal 2 , Gita Negi 3 , Yatendra Mohan 1 , Jyotsna Bhateja 1 Senior Resident 1 , Junior Resident 2 , Additional Professor and Head 3 , 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 INTRODUCTION Acquired hemophilia A (AHA) is a rare bleeding disorder seen in patients with no family history of hemophilia. It occurs due to the development of autoantibodies against endogenous factor VIII (FVIII). Clinically, it is similar to severe hemophilia A and is associated with spontaneous bleeds, which is usually mucocutaneous, soft tissue or gastrointestinal. Hemarthrosis is uncommon in AHA [1,2] . These bleeds are often difficult to predict and doesn’t correlate with the inhibitor titers or FVIII levels. The reported mortality is 9.7% to 33% [1–4] . It is associated with prolonged APTT which does not correct on addition of normal plasma. The diagnosis is confirmed from reduced FVIII levels and detectable FVIII inhibitor. It is much more common in adults [2,4,5] . FVIII inhibitors in AHA are mostly IgG1&4 autoantibodies following second- order kinetics and react with the same regions of the FVIII molecule (i.e., A2 and C2 domains) those targeted by FVIII alloantibodies [6] . The antibodies interfere with the coagulant activity of FVIII. Incidence The reported incidence of AHA is 1.34–1.48 cases per million per year [1] . Increasing age attributes to poor prognosis. Up to 85% of affected individuals are older adults (60 years) with no gender predilection [2] . The age distribution for