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

Mahmoud Marashi; Ahmed Aly Basha; Amar Lal; Hani Osman; Hasan Aal Yaseen; Hasan Ghazal; Sharukh Hashmi; Muhammad Khanani

doi:10.4103/joah.joah_25_22

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Year : 2022 | Volume : 13 | Issue : 4 | Page : 176-182

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

Mahmoud Marashi¹, Ahmed Aly Basha², Amar Lal³, Hani Osman³, Hasan Aal Yaseen¹, Hasan Ghazal⁴, Sharukh Hashmi⁵, Muhammad Khanani⁶
¹ Department of Hematology/Oncology, Dubai Hospital, Al Ain, UAE
² Department of Hematology/Oncology, Saudi German Hospital, Al Ain, UAE
³ Department of Hematology/Oncology, Tawam Hospital, Al Ain, UAE
⁴ Department of Hematology/Oncology, King's College Hospital, Al Ain, UAE
⁵ Department of Hematology/Oncology, Sheikh Shakhbout Medical, Al Ain, UAE
⁶ City Abu Dhabi, Pediatric Hematology/Oncology Department, Tawam Hospital, City Abu Dhabi, Al Ain, UAE

Correspondence Address:
Prof. Mahmoud Marashi
Dubai Hospital, Dubai
UAE

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/joah.joah_25_22

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.

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