title: Challenges and open issues in the management of acquired hemophilia A (AHA).
abstract: Acquired hemophilia A (AHA) is a rare autoimmune bleeding disorder caused by antibodies which neutralize the function of factor VIII (FVIII). The disease presents a complex clinical challenge to the treating Physicians and Hematologists. As the disease is associated with high mortality, prompt management is necessary. Early recognition, quick diagnosis and timely referral to a specialized center are important for better management of these patients. The different clinical manifestations, underlying pathology, inhibitor kinetics and the associated age related comorbidities do not allow extrapolation of the treatment protocols of congenital hemophilia to AHA. The basic strategies of the management of AHA patients involve maintaining hemostasis, suppression or eradication of antibodies, diagnosis and treatment of underlying pathology and avoid treatment related complications like thrombosis. The efficiency of hemostatic agents which are generally used to treat AHA is unpredictable. Due to the rarity of the disease, there are no randomized clinical trials on the management of this disorder and thus the expertise and experience of the treating Physicians' guide treatment strategies.
authors.affiliation: National Institute of Immunohaematology (ICMR), Department of Thrombosis and Haemostasis, 13th Floor, KEM Hospital, Parel, Mumbai, India. Electronic address: shrimatishetty@yahoo.com.
authors.forename: Shrimati D
authors.initials: SD
authors.lastname: Shetty
authors.affiliation: National Institute of Immunohaematology (ICMR), Department of Thrombosis and Haemostasis, 13th Floor, KEM Hospital, Parel, Mumbai, India.
authors.forename: Kanjaksha
authors.initials: K
authors.lastname: Ghosh
mesh_terms: D000818:Animals; D000933:Antifibrinolytic Agents / Q000627:therapeutic use; D001779:Blood Coagulation Factors / Q000627:therapeutic use; D015942:Factor VIIa / Q000627:therapeutic use; D006467:Hemophilia A / Q000175:diagnosis / Q000628:therapy*; D006490:Hemostatics / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D011994:Recombinant Proteins / Q000627:therapeutic use
chemical_list: D000933:Antifibrinolytic Agents; D001779:Blood Coagulation Factors; D006490:Hemostatics; D007166:Immunosuppressive Agents; D011994:Recombinant Proteins; C025667:prothrombin complex concentrates; C103587:recombinant FVIIa; D015942:Factor VIIa
keywords: Antifibrinolytics; Hemophilia; Immunosuppressives; Management; aPCC; rFVIIa