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EACH2: First-line immunosuppressive therapy did not affect survival in acquired hemophilia A
Although treatment with steroids plus cyclophosphamide resulted in a higher likelihood of complete remission, the choice of first-line immunosuppressive treatment did not affect final survival data among patients with acquired hemophilia A enrolled in the European Acquired Hemophilia Registry (EACH2), according to the results of a recently published study.
Although immunosuppression is recommended immediately after a diagnosis of acquired hemophilia A (AHA), the optimal treatment is still unknown. The goal of this study was to identify the optimal immunosuppressive regimen to treat the disease.
The EACH2 registry collected patient information from 2003 to 2009. During that time, researchers enrolled 294 patients who were treated with first-line immunosuppression with steroids alone (n=142), steroids plus cyclophosphamide (n=83) or rituximab-based regimens (n=51). The primary outcome was complete remission, which was defined as inhibitor undetectable, Factor VIII of 70 IU/dL or greater, and immunosuppression stopped.
Those patients assigned to steroids in addition to cyclophosphamide had more stable complete remission than those patients assigned to steroids alone (70% vs. 48%) or to rituximab-based regimens (59%). Patients assigned to steroids plus cyclophosphamide had a median time to complete remission of about 5 weeks, compared with 10 weeks for those assigned to rituximab-based regimens.
Only 16% of patients treated with steroids alone experienced an adverse event, most commonly infection. In comparison, 41% of patients assigned to the steroid and cyclophosphamide combination had an adverse event. Thirty-seven percent of patients treated with rituximab-based regimens reported an adverse event.
Final survival data was gathered after a median of 262 days, at which time 69% of patients were alive. At final follow-up, data indicated that the treatment assigned had no effect on survival among the groups, despite the differences in achieving stable complete remission.
“These data show that patients with AHA are more likely to achieve a stable remission after first-line therapy if treated with a combination of steroids and cyclophosphamide than with other regimens,” the researchers wrote. “Despite this, outcome at final follow-up is not affected by the choice of first-line therapy.”
For more information:
- Collins P. Blood. 2012;doi:10.1182/blood-2012-02-409185.