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International recommendations on the diagnosis and treatment of acquired hemophilia A

A. Tiede, P. Collins, P. Knoebl, J. Teitel, C. Kessler, M. Shima, G. Di Minno, R. d'Oiron, P. Salaj, V. Jiménez-Yuste, A. Huth-Kühne, P. Giangrande

. 2020 ; 105 (7) : 1791-1801. [pub] 20200507

Language English Country Italy

Document type Journal Article, Research Support, Non-U.S. Gov't

Persistent link   https://www.medvik.cz/link/bmc21020392

Acquired hemophilia A (AHA), a rare bleeding disorder caused by neutralizing autoantibodies against coagulation factor VIII (FVIII), occurs in both men and women without a previous history of bleeding. Patients typically present with an isolated prolonged activated partial thromboplastin time due to FVIII deficiency. Neutralizing antibodies (inhibitors) are detected using the Nijmegen-modified Bethesda assay. Approximately 10% of patients do not present with bleeding and, therefore, a prolonged activated partial thromboplastin time should never be ignored prior to invasive procedures. Control of acute bleeding and prevention of injuries that may provoke bleeding are top priorities in patients with AHA. We recommend treatment with bypassing agents, including recombinant activated factor VII, activated prothrombin complex concentrate, or recombinant porcine FVIII in bleeding patients. Autoantibody eradication can be achieved with immunosuppressive therapy, including corticosteroids, cyclophosphamide and rituximab, or combinations thereof. The median time to remission is 5 weeks, with considerable interindividual variation. FVIII activity at presentation, inhibitor titer and autoantibody isotype are prognostic markers for remission and survival. Comparative clinical studies to support treatment recommendations for AHA do not exist; therefore, we provide practical consensus guidance based on recent registry findings and the authors' clinical experience in treating patients with AHA.

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$a Acquired hemophilia A (AHA), a rare bleeding disorder caused by neutralizing autoantibodies against coagulation factor VIII (FVIII), occurs in both men and women without a previous history of bleeding. Patients typically present with an isolated prolonged activated partial thromboplastin time due to FVIII deficiency. Neutralizing antibodies (inhibitors) are detected using the Nijmegen-modified Bethesda assay. Approximately 10% of patients do not present with bleeding and, therefore, a prolonged activated partial thromboplastin time should never be ignored prior to invasive procedures. Control of acute bleeding and prevention of injuries that may provoke bleeding are top priorities in patients with AHA. We recommend treatment with bypassing agents, including recombinant activated factor VII, activated prothrombin complex concentrate, or recombinant porcine FVIII in bleeding patients. Autoantibody eradication can be achieved with immunosuppressive therapy, including corticosteroids, cyclophosphamide and rituximab, or combinations thereof. The median time to remission is 5 weeks, with considerable interindividual variation. FVIII activity at presentation, inhibitor titer and autoantibody isotype are prognostic markers for remission and survival. Comparative clinical studies to support treatment recommendations for AHA do not exist; therefore, we provide practical consensus guidance based on recent registry findings and the authors' clinical experience in treating patients with AHA.
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$a Collins, Peter $u Arthur Bloom Haemophilia Centre, University Hospital of Wales School of Medicine, Cardiff University, Cardiff, UK
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$a Knoebl, Paul $u Department of Medicine 1, Division of Hematology and Hemostasis, Medical University of Vienna, Vienna, Austria
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$a Teitel, Jerome $u Division of Hematology and Oncology, St. Michael's Hospital, Toronto, and Department of Medicine, University of Toronto, Toronto, Canada
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$a Kessler, Craig $u Georgetown University Hospital, Lombardi Cancer Center, Division of Hematology/Oncology, Washington, DC, USA
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$a Shima, Midori $u Department of Pediatrics, Nara Medical University, Nara, Japan
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$a Di Minno, Giovanni $u Regional Reference Center for Coagulation Disorders, Federico II University Hospital, Naples, Italy
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$a d'Oiron, Roseline $u Centre de Référence de l'Hémophilie et des Maladies Hémorragiques Constitutionnelles Rares, Hôpitaux Universitaires Paris Sud, Hôpital Bicêtre APHP, Le Kremlin-Bicêtre, France
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$a Salaj, Peter $u Institute of Hematology and Blood Transfusion, Prague, Czech Republic
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$a Jiménez-Yuste, Victor $u Hematology Department, La Paz University Hospital, Autonoma University, Madrid, Spain
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$a Huth-Kühne, Angela $u SRH Kurpfalzkrankenhaus Heidelberg GmbH and Hemophilia Center, Heidelberg, Germany
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$a Giangrande, Paul $u Green Templeton College, University of Oxford, Oxford, UK
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