Successful management of acquired hemophilia A with life-threatening bleeding and MRSA bacteremia in a hemodialysis patient: importance of a multimodal approach

Background Acquired hemophilia A (AHA) is a rare and fatal bleeding disorder. AHA in patients undergoing hemodialysis (HD) often leads to impaired hemostasis, necessitating temporary catheters that increase the risk of catheter-related infection. This complication hinders the use of immunosuppressive therapy, often resulting in treatment failure. We herein report the case of AHA in a patient undergoing HD with life-threatening bleeding and MRSA bacteremia, which was successfully managed with a multimodal approach. Case presentation A 64-year-old Japanese man had undergone HD owing to diabetic nephropathy and was admitted to our hospital with a massive hematoma in the left iliopsoas muscle and poor hemostasis at the vascular access site. Hemoglobin (Hb) had dropped to 5.0 g/dL despite daily blood transfusions, and activated partial thromboplastin time was alone prolonged around 60.0 s. Transcatheter arterial embolization (TAE) was performed owing to difficulty in hemostasis. After the diagnosis of AHA was made, 60 mg/day of prednisolone and bypassing agents were introduced. Additional plasma exchange (PE) was performed and rituximab was also administered. Factor VIII (FVIII) inhibitor decreased from 91.0 to 32.0 Bethesda Units (B.U)/mL, and hemostasis was achieved temporarily. Despite a decreasing trend, the FVIII inhibitor titer persisted at a high range. Hb decreased to 5.7 g/dL, and blood transfusions were needed again. Thus, a second TAE and a third PE were performed. After that, FVIII inhibitor improved to 7.0 B.U/mL, and hemostasis was fully achieved. Notably, a temporary dialysis catheter led to methicillin-resistant Staphylococcus aureus (MRSA) bacteremia, necessitating careful PSL tapering. At 6 months after discharge, the patient had not experienced any relapse. Conclusions AHA in patients undergoing HD often leads to difficulty in hemostasis during HD, necessitating temporary catheters that almost induce catheter-related infection. This complication hinders the use of immunosuppressive therapy, sometimes resulting in treatment failure. Given the specific clinical setting of AHA in patients undergoing HD, achieving rapid bleeding control and preparing for impending infections are paramount, which necessitates a well-designed multimodal approach including immunosuppressive therapy and PP (plasmapheresis). This instructive case offers a potential framework for managing similar high-risk patients.