Background: Simultaneous pancreas-kidney transplantation (SPK) is a definitive treatment for end-stage kidney disease (ESKD) and type 1 diabetes mellitus. However, even with careful perioperative control of hemostasis, SPK carries a high risk of postoperative bleeding, which is exacerbated in patients with hemophilia A. Here, we report the first known successful SPK in a patient with congenital hemophilia A.
Case report: A 52-year-old man was diagnosed with hemophilia A at 2 years of age. He developed type 1 diabetes mellitus at 25 years of age, which progressed to ESKD at 43 years of age. A comprehensive preoperative FVIII replacement protocol was applied during the perioperative period, using continuous infusion with bolus supplementation to maintain target VIII activity levels (100% intraoperatively, 80% during the first postoperative week, and ≥30% thereafter).
Conclusion: This case demonstrates that SPK transplantation can be safely performed in patients with congenital hemophilia A, by implementing appropriate FVIII replacement strategies and close multidisciplinary collaboration.
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