Combined Immunosuppression for Acquired Hemophilia A: CyDRi is a Highly Effective Low Toxicity Regimen

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 AHA patients treated on an identical institutional such protocol: 1000 mg cyclophosphamide on day 1 and 22, 40 mg dexamethasone on day 1, 8, 15 and 22, and 100 mg rituximab on day 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, IQR 2.5-29.5). 31/32 (96.8%) of patients achieved durable CR. 29/32 patients (90.6%) were alive at last follow up, all of them in CR. The median time to reach first CR was 77 days (range 14-939; IQR 31-115). Toxicity and side effects were acceptable and milder than commonly used, prolonged steroid therapies. In conclusion, the CyDRi regimen produced markedly higher complete remission rates and overall survival than currently used sequential regimens. Taken together, CyDRi proved to be an attractive option for the immunosuppression of elderly AHA patients.