The rare severe autoimmune bleeding illness known as acquired hemophilia A (AHA) has a high morbidity and fatality rate. Despite its importance in disease management, there is no agreement on the ideal immunosuppressive regimen.
Steroids are often used as a first line, with additional medicines used if steroid usage fails. The advantage of upfront combination regimens was that they boosted effectiveness while reducing steroid exposure and toxicity. Data from 32 AHA patients who received the same institutional treatment protocol of cyclophosphamide 1,000 mg on days 1 and 22 and dexamethasone 40 mg on days 1, 8, and 15, as well as rituximab 100 mg on days 1, 8, and 22 were retrospectively examined (the regimen was termed CyDRi). CyDRi was given to all patients for at least one cycle. CyDRi was repeated up to day 43 of the preceding cycle, if necessary, until remission. Control of the bleeding was quickly attained.
The average amount of time to stop bleeding was 15.5 days (range, 0-429 days; interquartile range, 2.5-29.5 days). About 31 patients (96.8%) experienced durable complete remission (CR); 29 patients (90.6%) were still alive at the time of the final follow-up, all of whom were in CR. It took 77 days on average to reach the first CR (range, 19-939 days; interquartile range, 31-115 days). The toxicity and side effects were tolerable and less severe than with conventional, extended steroid therapy.
In conclusion, the CyDRi regimen showed noticeably greater CR rates and overall survival compared to presently employed sequential regimens. CyDRi emerged as a desirable choice for the immunosuppression of older individuals with AHA when taken as a whole.
