International Recommendations on the Diagnosis and Treatment of Acquired Hemophilia A

Overview

Journal Haematologica

Specialty Hematology

Date 2020 May 9

PMID 32381574

Citations 124

Authors

Andreas Tiede

Peter Collins

Paul Knoebl

Jerome Teitel

Craig Kessler

Midori Shima

Giovanni Di Minno

Roseline dOiron

Peter Salaj

Victor Jimenez-Yuste

Angela Huth-Kuhne

Paul Giangrande

Affiliations

Soon will be listed here.

Abstract

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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