Diagnosis and Management of Acquired Hemophilia A: Case Reports and a Literature Review

Abstract

Background. Acquired hemophilia A (AHA) is a potentially life-threatening autoimmune hemostatic disorder where autoantibodies that disrupt the functions of factor VIII (FVIII) are present in the circulation. The early diagnosis of AHA is difficult since the symptoms of AHA differ from those of congenital hemophilia A. Furthermore, the management of AHA is also more complex due to the presence of autoantibodies against FVIII (FVIII inhibitors). Here, we present three case reports and conduct a literature review of AHA with the aim to increase awareness and knowledge regarding the diagnosis and treatment of AHA. Case Presentations. We present three patients diagnosed with AHA in these case reports. The first patient was a young female, while the second and third patients were middle-aged and elderly males, respectively. All patients presented with a chief complaint of bruises without hemarthrosis and a history of bleeding. Laboratory examinations of the patients revealed isolated prolonged aPTT, normal PT, and the presence of autoantibodies against factor VIII, which are characteristics of AHA. Patients were then treated with corticosteroids to reduce the titer level of autoantibodies and received factor VIII transfusion to stop bleeding. Conclusion. AHA can be suspected in patients presenting with symptoms of bruises without hemarthrosis and without the history of bleeding. Isolated aPTT elevation with normal PT should raise high suspicion of AHA. The presence of FVIII inhibitors can help to confirm the diagnosis of AHA. Treatment consists of factor VIII transfusion and corticosteroid therapy. Bypassing agents are recommended as an alternative to FVIII transfusion.

Acquired hemophilia A following COVID-19 vaccine: a case report

“…Treatment is focused on symptomatically treating any bleeding along with etiological treatment. In urgent situations such as uncontrolled bleeding, the administration of recombinant FVII activated or the activated prothrombin complex can help control bleeding [ 9 , 14 ]. The main treatment of AHA focuses on inhibitor eradication relying on immunosuppressive agents such as corticosteroids, rituximab and cyclophosphamide [ 15 , 16 ].…”

Diagnosis and Management of Post-Partum Hemorrhage Caused by Acquired Hemophilia A: A Case Report

“…The hemostatic arm applies for patients with severe bleeding. Interestingly, because AHA antibodies could be non-neutralizing, some AHA patients benefit from continuous administration of FVIII concentrates, specifically if the inhibitors level are low (＜5 BU) [12].…”

“…The immunosuppression arm uses steroids (prednisone, 1 mg/kg/day for 4∼ 6 weeks), with multiple immunosuppressants (eg, cyclophosphamide cyclosporine, azathioprine, 6-mercaptopurine, and vincristine). The main treatment lines that have been used recently includes corticosteroid alone, corticosteroid with cyclophosphamide, and corticosteroids with rituximab [12].…”

Penicillin-Induced Anti-Factor VIII Antibodies: A Case Report

“…In addition, the symptoms are not associated with the levels of factor VIII. 3,4 From the therapeutic point of view, corticoids and also immunosuppressants such as cyclosporine, vincristine, and cyclophosphamide have been used in medication with the purpose of inactivating and preventing coagulation in patients with acquired hemophilia. In addition, innovative therapies with immunoglobulin, interferon, plasmapheresis, and even rituximab have actually been attempted, although a conventional therapy has not yet been found.…”

“…In addition, innovative therapies with immunoglobulin, interferon, plasmapheresis, and even rituximab have actually been attempted, although a conventional therapy has not yet been found. 5,6 In this paper, we reported the case of an individual that acquired hemophilia after the use of benzathine penicillin for the therapy of syphilis complicated with joint hemarthrosis. Although there have been previous reports of cases of acquired hemophilia induced by penicillin with spontaneous remission or with a high complete response rate to immunosuppression with the use of corticoid alone.…”