PB1196 - Clinical Characteristics and Treatment Outcomes of Acquired Hemophilia A at A National Referral Hospital in Indonesia

Background: Acquired hemophilia A (AHA) is a rare bleeding disorder with significant morbidity and mortality, caused by the development of autoantibodies against factor VIII (FVIII). Bypassing agents, as the treatment of choice of AHA, are not always available in Indonesia, which may contribute to the outcomes of patients.

Aims: To present the clinical characteristics and treatment outcomes of 20 AHA cases at a referral hospitals in Indonesia.

Methods: We retrospectively reviewed AHA cases at Cipto Mangunkusumo General Hospital, Jakarta, Indonesia, over a period of 10 years. The inclusion criteria were subjects with clinical bleeding and prolonged APTT, FVIII level < 0.4 U/mL with/without FVIII inhibitor, and previously normal hemostasis.

Results: The median age of AHA at diagnosis was 60 years old, predominantly female (70%). Most subjects had one or multiple associated conditions, with severe (65%) or moderate (30%) FVIII deficiency, and high (35%) and very high (35%) inhibitor titers (Table 1). The most common bleeding manifestation was severe cutaneous bleeding, followed by gastrointestinal, joint, and muscle bleeding. Bleeding was controlled using plasma-derived FVIII (65%), activated prothrombin complex concentrate/aPCC (25%), and prothrombin complex concentrate/PCC (15%). Cryoprecipitate (35%) and fresh frozen plasma/FFP (15%) were administered prior to AHA diagnosis (Table 2). Fourteen (70%) subjects were treated with immunosuppressive agents, either corticosteroid alone (45%) or in conjunction with other immunosuppressive agents (25%), and both yielded high overall response rate (88.8% vs 80%). Bleeding subsided in 18 subjects, generally after one to three weeks of treatment.

Conclusion(s): AHA commonly develops in the elderly and is associated with severe cutaneous bleeding and severe FVIII deficiency. The underlying conditions vary. Bypassing agents (PCC, aPCC) were used in 40% patients. AHA with high or very high inhibitor titers responded well to immunosuppressive agents.