Management of acquired hemophilia: Consensus recommendations for the United Arab Emirates practitioners

Abstract

Acquired hemophilia A (AHA) is a rare autoimmune condition characterized by spontaneous antibodies formation against coagulation factor VIII (FVIII). In the United Arab Emirates (UAE), the treatment paradigm of AHA varies, leading to heterogeneous patient outcomes. Because AHA cases often present to clinical practitioners with insufficient experience in the disease, diagnosis is sometimes delayed. This document aims to provide recommendations to clinical practitioners in the UAE to bridge the gap of heterogeneous practice in the clinical management of AHA. Structured literature searches in PubMed and Google Scholar with a three-stage Delphi method were conducted. Evidence suggests that prolonged activated partial thromboplastin time before surgery or coupled with acute or recent onset of bleeding is a hallmark of AHA. Treatment using bypassing agents is recommended in AHA patients with bleeding. Corticosteroids and rituximab with (out) a cytotoxic agent represent the first-line treatment in patients with FVIII: C <1 IU/dL. Second-line therapy involves agents not utilized in the first round of treatment. Thromboprophylaxis is recommended if FVIII: C has returned to normal levels. These recommendations are intended to improve the clinical practice and awareness of this disorder among hematologists and nonhematologists in the UAE and provide practical diagnosis and treatment advice.

Keywords

• acquired hemophilia a
• activated partial thromboplastin time
• factor viii