SUCCESSFUL HEMODIALYSIS INITIATION IN ACQUIRED HEMOPHILIA A MANAGED WITH SUSOCTOCOG ALFA AND EMICIZUMAB

Acquired hemophilia A (AHA) is a rare, life-threatening bleeding disorder caused by factor VIII inhibitors. In patients who require hemodialysis (HD), bleeding from vascular access and anticoagulation create unique hazards, making it difficult to safely initiate or maintain HD.

Evidence supporting recombinant porcine FVIII (susoctocog alfa) for acute hemostasis and emicizumab for prophylaxis is accumulating, but real-world guidance on how to sequence these agents during HD initiation remains limited.

We managed an 83-year-old man with nephrosclerosis, recurrent pneumonia, nephrotic-range proteinuria (selectivity index 0.16), hypoalbuminemia, and progressive renal failure. On admission, activated partial thromboplastin time (APTT) was prolonged despite no bleeding history. Because of pulmonary disease and coagulopathy, renal biopsy was deferred and prednisolone (30 mg/day, 0.5 mg/kg/day) was initiated for suspected minimal-change nephrotic syndrome. As renal function deteriorated, HD via a temporary catheter was started. Persistent oozing from the catheter site prompted coagulation studies: the mixing test showed an inhibitor pattern and low FVIII activity, leading to a diagnosis of AHA. Despite ongoing bleeding under steroid therapy, intensification of immunosuppressive therapy (IST) was avoided due to advanced age, renal failure, and infection risk. Hemostatic therapy was prioritized to safely continue HD.

Initial hemostasis with recombinant activated factor VII (rFVIIa; eptacog alfa) was insufficient. Susoctocog alfa promptly normalized APTT and controlled access-site bleeding, enabling long-term catheter placement and initiation of maintenance HD without procedure-related hemorrhage. After HD stabilization, emicizumab was introduced for prophylaxis (270 mg on Day 48, 135 mg on Day 49, then 60 mg weekly from Day 55). No further significant bleeding occurred during maintenance HD, although the escalation of IST was still avoided due to recurrent respiratory infections.

We report a rare case of nephrotic syndrome complicated by AHA in which maintenance HD was successfully initiated and sustained without intensifying IST, given the patient’s high infection risk. The patient developed bleeding while receiving prednisolone 0.5 mg/kg/day, and diagnostic testing confirmed AHA. Hemostasis with eptacog alfa was insufficient, whereas susoctocog alfa promptly achieved bleeding control, enabling long-term catheter placement and safe initiation of HD. Subsequent emicizumab prophylaxis maintained hemostatic stability and allowed continued HD without escalation of IST. Although a Th2/IgG4-skewed immune background could mechanistically link nephrotic syndrome and AHA and the concurrence of both disease were previously reported , this relationship remains speculative. To our knowledge, this is the first report documenting HD initiation and maintenance in AHA using the sequential susoctocog alfa followed by emicizumab. This case highlights an infection-conscious practical strategy for frail patients-prioritizing rapid procedural hemostasis with rpFVIII when rFVIIa response is inadequate, followed by emicizumab prophylaxis to sustain bleed prevention and minimize steroid or IST exposure.