ArticleHaematologica2020
International recommendations on the diagnosis and treatment of acquired hemophilia A.
Article in Haematologica, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 208 papers, 3 of them syntheses that pooled it.
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Who cites it
208 citing papers in PubMed, 3 syntheses or guidelines pooled it, 356 citations in OpenAlex.
- Acquired hemophilia A following SARS-CoV-2 infection and vaccination: clinical summary and insights.Journal of thrombosis and haemostasis : JTH · 2026Pooled it
- Updated Egyptian national guidelines for management of hemophilia A in children & adolescents.Annals of hematology · 2025Guideline
- Acquired Hemophilia A after SARS-CoV-2 Infection: A Case Report and an Updated Systematic Review.Biomedicines · 2023Pooled it
- Sustained survival benefit of emicizumab and postponed immunosuppression in acquired hemophilia A.Blood advances · 2025Trial
- Final Analysis Results from the AGEHA Study: Emicizumab Prophylaxis for Acquired Hemophilia A with or without Immunosuppressive Therapy.Thrombosis and haemostasis · 2025Trial
- Efficacy and safety of recombinant porcine factor VIII in Japanese patients with acquired hemophilia A.International journal of hematology · 2024Trial
- Cost-effectiveness analysis of emicizumab use in hospitalized older individuals with acquired hemophilia A.Blood vessels, thrombosis & hemostasis · 2026Article
- How I Investigate Borderline Prolonged aPTT: An Integrated Laboratory Approach to Mixing Studies and Factor Analysis.International journal of laboratory hematology · 2026Review
- When A Fall Isn't Just a Fall: Delayed Diagnosis of Acquired Hemophilia A in a Nonagenarian Patient.The American journal of case reports · 2026Article
- Acute Groin Pain Revealing Acquired Hemophilia A: A Case Report of Spontaneous Iliopsoas Hematoma.Cureus · 2026Article
- Acquired hemophilia A following wasp stings: a case report.World journal of emergency medicine · 2026Article
- Real-world Diagnosis and Testing of Acquired Hemophilia A in Japan: A Web-based Physician Survey.Internal medicine (Tokyo, Japan) · 2026Observational
- Sequential Use of Recombinant Porcine Factor VIII and Early Emicizumab in Acquired Hemophilia A: A Case Series.EJHaem · 2026Article
- Acquired Hemophilia A Revealing Occult Splenic Marginal Zone Lymphoma.The American journal of case reports · 2026Article
- Acquired hemophilia A: an illustrated review based on the French National Guidelines.Research and practice in thrombosis and haemostasis · 2026Review
- Severe Acquired Factor VIII Deficiency With Concomitant Lupus Anticoagulant: A Case Report.Cureus · 2026Article
- Hematoma, Hemarthrosis, and Hemotympanum: A Case Report of Acquired Hemophilia A.Journal of education & teaching in emergency medicine · 2026Article
- Acquired hemophilia A following influenza vaccination: a case report and literature review.Annals of hematology · 2026Review
- Refractory Hemorrhage Following Total Knee Replacement Unmasking Acquired Hemophilia A.Cureus · 2026Article
- Delayed diagnosis of acquired hemophilia a complicating hepatocellular carcinoma: a rare case of hepatectomy following atezolizumab and bevacizumab therapy.Clinical journal of gastroenterology · 2026Article
148 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
12 authors at 11 institutions in 10 countries.
Funding
No grant is acknowledged in the PubMed record.
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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