ArticleBlood advances2021
Management of acquired hemophilia A: results from the Spanish registry.
Article in Blood advances, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Who cites it
19 citing papers in PubMed, 37 citations in OpenAlex.
- Sustained survival benefit of emicizumab and postponed immunosuppression in acquired hemophilia A.Blood advances · 2025Trial
- Acquired hemophilia A: an illustrated review based on the French National Guidelines.Research and practice in thrombosis and haemostasis · 2026Review
- Incidence of infections in patients treated with rituximab for autoimmune disorders of hematological Interest or non-Hodgkin lymphoma.Annals of hematology · 2026Article
- Gene Therapy in Hemophilia: Clinical Advances, Immunological Challenges, and Emerging Therapeutic Perspectives.International journal of molecular sciences · 2026Review
- Infectious complications in acquired hemophilia A: insights from the Spanish registry (AHASR).Haematologica · 2026Article
- [How I treat acquired clotting factor deficiency].Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2025Article
- Standard dose and low-dose rituximab combined with glucocorticoids and cyclophosphamide in the treatment of acquired haemophilia A.Frontiers in immunology · 2025Article
- Comorbidity and adverse events in acquired hemophilia A: data from the GTH-AHA-EMI study.Research and practice in thrombosis and haemostasis · 2024Article
- A Rare Case of Acquired Factor VIII Inhibition in an Elderly Female.Journal of community hospital internal medicine perspectives · 2024Article
- Acquired hemophilia A: a single-center study of 165 patients.Research and practice in thrombosis and haemostasis · 2024Article
- Immunotherapy of acquired hemophilia A.Hematology. American Society of Hematology. Education Program · 2023Article
- Key Genes of the Immune System and Predisposition to Acquired Hemophilia A: Evidence from a Spanish Cohort of 49 Patients Using Next-Generation Sequencing.International journal of molecular sciences · 2023Article
- Systemic lupus erythematosus with acquired hemophilia A: A case report and literature review.Zhong nan da xue xue bao. Yi xue ban = Journal of Central South University. Medical sciences · 2023Review
- Combining Low-Dose Rituximab and Bortezomib as Immunosuppressive Therapy for Acquired Hemophilia A: 6 Case Series.Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion · 2023Article
- Combined immunosuppression for acquired hemophilia A: CyDRi is a highly effective low-toxicity regimen.Blood · 2022Article
- Acquired haemophilia A: Italian Consensus Recommendations on diagnosis, general management and treatment of bleeding.Blood transfusion = Trasfusione del sangue · 2022Article
- Acquired Haemophilia A: A Review of What We Know.Journal of blood medicine · 2022Review
- Acquired Hemophilia A: A Retrospective Multicenter Analysis of 42 Patients.Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/HemostasisArticle
- Real-World Use of Emicizumab in Patients With Acquired Haemophilia A: An Interim Safety Analysis of a Large-Scale Post‑Marketing Surveillance Study.Haemophilia : the official journal of the World Federation of HemophiliaArticle
Corrections and comments
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Authors and funding
38 authors at 20 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The Spanish Acquired Hemophilia A (AHA) Registry is intended to update the status of AHA in Spain. One hundred and fifty-four patients were included and retrospectively followed for a median of 12 months. Patients were predominantly male (56.3%), with median age at diagnosis of 74 years. AHA was more frequently idiopathic (44.1%) and autoimmune disorder-associated (31.7%). Thirty-four percent of patients were on antithrombotic therapy at diagnosis. Hemostatic treatment was used in 70% of patients. Recombinant activated factor VII was more frequently infused (60.3% vs 20.6% activated prothrombin complex concentrate). Only 1 patient did not achieve control of hemorrhage. Complete remission (CR) was achieved by 84.2% of cases after immunosuppressive therapy. Steroids alone were less efficient than the other strategies (68.2% vs 87.2%, P = .049), whereas no differences existed among these (steroids/cyclophosphamide, 88.5%, vs steroids/calcineurin inhibitors, 81.2%, vs rituximab-based regimens, 87.5%). Female sex and high inhibitor levels influenced CR negatively. Thirty-six deaths (23.8%) were reported. Main causes of death were infection (15 patients, 9.9%) and hemorrhage (5 patients, 3.3%). All hemorrhage-related and half the infection-related deaths occurred within 2 months of diagnosis. Prior antithrombotic therapy was inversely associated with survival, irrespective of age. Median age of nonsurvivors was significantly higher (79 vs 73 years in survivors). Patients dying of infection were older than the other nonsurvivors (85 vs 78 years). In summary, fatal infection in the first months is common in our series. Antithrombotic therapy is associated with mortality. Particular care should be taken to avoid misdiagnosis.
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