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.
