ArticleBlood2022
Combined immunosuppression for acquired hemophilia A: CyDRi is a highly effective low-toxicity regimen.
Article in Blood, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed, 30 citations in OpenAlex.
- Sustained survival benefit of emicizumab and postponed immunosuppression in acquired hemophilia A.Blood advances · 2025Trial
- Postpartum acquired hemophilia A: immunopathogenesis, diagnostic challenges, and treatment with the CyDRi protocol.Pathology oncology research : POR · 2026Review
- Deferral of immunosuppression in acquired hemophilia.Blood advances · 2025Article
- Prognostic factors for recurrence in acquired hemophilia A-results from a long-term observational study.Research and practice in thrombosis and haemostasis · 2025Article
- Acquired hemophilia A as a disease of the elderly: A comprehensive review of epidemiology, pathogenesis, and novel therapy.GeroScience · 2025Review
- Standard dose and low-dose rituximab combined with glucocorticoids and cyclophosphamide in the treatment of acquired haemophilia A.Frontiers in immunology · 2025Article
- Real-world impact of emicizumab and immunosuppression on acquired hemophilia A: a multicenter US cohort.Blood advances · 2024Article
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- Case Report: Effective management of adalimumab-induced acquired hemophilia A with the CyDRI protocol.Pathology oncology research : POR · 2024Article
- Immunotherapy of acquired hemophilia A.Hematology. American Society of Hematology. Education Program · 2023Article
- Delayed spontaneous remission of acquired factor V inhibitor refractory to immunosuppressive therapy with pregnancy-associated improvement.Pathology oncology research : POR · 2023Article
- 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
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Authors and funding
13 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Acquired hemophilia A (AHA) is a rare severe autoimmune bleeding disorder with significant morbidity and mortality. Although critical for disease control, there is no consensus for the best immunosuppressive regimen. Most authors use steroids first line, followed by other agents for steroid failures. Upfront combined regimens offer the advantage of reduced steroid exposure and toxicity as well as increased efficacy. We retrospectively analyzed data from 32 patients with AHA treated on an identical such institutional protocol: cyclophosphamide 1000 mg on days 1 and 22, dexamethasone 40 mg on days 1, 8, 15, and 22, and rituximab 100 mg on days 1, 8, 15, and 22 (the regimen was termed CyDRi). All patients received at least 1 cycle of CyDRi. If necessary, CyDRi was repeated until remission, no sooner than day 43 of the previous cycle. Bleeding control was rapidly achieved. The median time for bleeding control was 15.5 days (range, 0-429 days; interquartile range, 2.5-29.5 days). Thirty-one (96.8%) of 32 patients achieved durable complete remission (CR); 29 (90.6%) of 32 patients were alive at last follow-up, all of them in CR. The median time to reach first CR was 77 days (range, 19-939 days; interquartile range, 31-115 days). Toxicity and side effects were acceptable and milder than those of commonly used, prolonged steroid therapies. In conclusion, the CyDRi regimen produced markedly higher CR rates and overall survival than currently used sequential regimens. Taken together, CyDRi proved to be an attractive option for the immunosuppression of elderly patients with AHA.
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