Relapse Does Not Affect Survival in Patients with Acquired Hemophilia A, Study Suggests

The relapse rate is higher in older patients with acquired hemophilia A (AHA) treated with immunosuppressive therapies, but it doesn’t affect their overall survival compared with those who don’t relapse, a study suggests.

The study, “Relapse pattern and long‐term outcomes in subjects with acquired haemophilia A,” was published in the journal Haemophilia.

AHA is a bleeding disorder caused by an autoimmune attack on the blood clotting factor VIII (FVIII). Immunosuppressive treatments are used to prevent acute hemorrhage and control the bleeding by eliminating the autoantibodies generated by the body.

Patients are known to relapse even after reaching complete remission with immunosuppressive treatments. However, the long-term history for these patients and factors that predispose them to relapse have not been studied in depth.

Here, Canadian researchers evaluated the medical history records of patients with AHA to identify relapse patterns, risk factors that contribute to re-occurrence of the condition, and these factors’ influence on patient survival.

Using the Quebec Reference Centre for Inhibitors (QRCI) database, the records of 111 hemophilia A patients were identified for use in the study. In Quebec, Canada, all hemophilia A patients requiring immunosuppressive treatment have been referred to the QCRI since 2000. Its database holds the medical history and treatment information for all treated patients.

Researchers reviewed medical cases at QCRI from January 2000 to December 2012. The median age of the study group was 73 years old with a median follow-up period of 25.6 months. With treatment, 90% of the patients achieved remission within a median period of 45 days. The patients were divided into two groups: those who relapsed (71% men) and those with no history of relapse (45% men).

The median age of the patients who relapsed was 78 years compared with 72 years in those without a relapse episode.

Researchers found that 14% of the patients relapsed at least once within a median of 13.4 months. Most relapses responded well to treatment and continued to be monitored.

They assessed the levels of FVIII and its inhibitor at the time of diagnosis to see whether they predicted a relapse and found that there was no significant correlation. Similarly, the type of immunosuppressive treatment regimen given to the patient also did not predict relapse.

Of the total cases included in the study, 53 AHA patients had an underlying condition. According to statistical analysis, associated lymphoproliferative syndrome (LPS) was the only underlying condition that could predict a relapse.

The mortality rate in patients who relapsed was 43%, whereas in those who did not, it was 31%. Despite the difference in their mortality rate, 60% of the patients in both groups were estimated to be alive five years after initial diagnosis, showing that the groups’ overall survival rate was similar.

“The long‐term follow‐up of our patients also showed that late or multiple relapses may occur, but that relapse is not associated with a worse [overall survival]. Thus, long‐term follow‐up is important for optimal management of AHA,” the study concluded.