A study published in Haemophilia suggested that older age, male gender, and associated lymphoproliferative syndromes (LPS) may predispose patients with acquired hemophilia A (AHA) to relapse. Furthermore, relapse did not appear to be linked to a worse prognosis, and immunosuppressive agents may be effectively repeated in elderly patients.
Investigators assessed long-term follow-up data from 111 patients with AHA who were followed for a median of 25.6 months. All patients were treated between 2000 and 2012 at the Quebec Reference Centre for Inhibitors in Canada. The researchers examined factors predictive of AHA relapse including plasma factor VIII and inhibitor levels, immunosuppressive and hemostatic regimens, and time to remission, relapse, or death. They also studied the influence of relapse on survival.
An underlying disorder such as autoimmune disease or LPS was present in 53 patients, and 11 patients died before reaching remission. Among the remaining patients, median time to remission was 45 days. During the observation period, 14 patients presented with 1 or more relapses, with a median time from diagnosis to first relapse of 13.4 months. Immunosuppressive treatment was successful in most of the relapse episodes, allowing second remission or further to be reached in 12 of the 14 patients. Mortality rates were 31% and 43% (P =.377) for patients with and without relapse, respectively.
Associated LPS was found to be predictive of relapse (P =.008), but factor VIII activity at baseline, inhibitor titer at initial diagnosis, and immunosuppressive regimen were not. Most patients who relapsed were male and older, but these variables were not statistically significant predictors for relapse.
The authors noted that their findings are limited by their retrospective nature but suggested that certain patients may experience late or multiple relapses, making long‐term follow‐up paramount for optimal management.
1. Mizrahi T, Doyon K, Dubé E, et al. Relapse pattern and long‐term outcomes in subjects with acquired haemophilia A [published online January 29, 2019]. Haemophilia. doi: 10.1111/hae.13685