[Acquired hemophilia A with reappearance of inhibitor 10 years after the completion of immunosuppressive therapy].

ABSTRACT

Acquired hemophilia A (AHA) is a disease that causes severe bleeding with the appearance of an inhibitor (INH) against blood coagulation factor VIII (FVIII). The prevalence of this condition is low; it occurs in only one in one to four million people per year; however, the number of diagnosed cases has increased in recent years owing to the greater awareness of the disease. It is noteworthy that this is a hemorrhagic disease that suddenly develops in the elderly. AHA treatment is divided into hemostatic treatment for bleeding and immunosuppressive therapy (IST) for removing FVIII-INH. As long as FVIII-INH remains, there is a risk of fatal bleeding; therefore, it is desirable to start IST soon after diagnosis. However, the use of immunosuppressive drugs for the elderly is often challenging due to concerns about adverse events, such as infectious diseases that have a considerable impact on prognosis. Ten years after the end of IST, we managed the case of a patient with AHA who had a relapse of FVIII-INH at the age of 84 years. In this case, relapse was detected early when there was no bleeding symptom, and remission was rapidly achieved with a small amount of IST without any adverse effects. There are few reports on AHA relapse; we believe that the present report will contribute meaningfully to the literature on this subject and would be useful when considering the long-term management of AHA.