#3233 Renal replacement therapy in a patient with severe hemophilia A and ESRD: a case report

Abstract Background and Aims Selecting an appropriate renal replacement therapy (RRT) for patients with end-stage renal disease (ESRD) and severe hemophilia A is complex due to the high risk of bleeding and vascular access complications. Peritoneal dialysis (PD) is often preferred to avoid systemic anticoagulation, but it may not always be feasible. Hemodialysis (HD) requires careful hemostatic management, particularly regarding vascular access. This case report highlights the decision-making process and challenges in choosing the optimal RRT modality for a patient with severe hemophilia A and ESRD. Methods A 55-year-old male with severe hemophilia A (Factor VIII activity <1%) and insulin-dependent diabetes mellitus developed ESRD requiring RRT. Severe hemophilic arthropathy limited mobility, ruling out PD. Lack of social support further excluded home-assisted PD. AVF creation was not a viable option, as the patient was set to begin chronic hemodialysis at a regional center with limited experience in hemophilia management and, crucially, an unstable supply of recombinant Factor VIII (rFVIII), essential for controlling prolonged bleeding from the AVF. Consequently, a tunneled central venous catheter was placed under prophylactic rFVIII administration. Post-procedure bleeding at the catheter site was successfully managed with additional rFVIII and hemostatic sutures. HD was performed without systemic anticoagulation, using saline flushes to maintain circuit patency. Results Over nine months, the patient underwent 111 HD sessions, achieving an average Kt/V of 1.1. No major bleeding or thrombotic complications occurred. Heparin was avoided during HD, and post-dialysis administration of 2000 units of rFVIII minimized bleeding risk. Four episodes of intradialytic hypotension were recorded. The patient was planned for transition to emicizumab to enhance long-term hemostatic stability. Unfortunately, he passed away at home, likely due to sudden cardiac death. Conclusions This case underscores the challenges of RRT selection in patients with ESRD and severe hemophilia A. While PD avoids vascular access complications, it requires patient capability and adequate support. AVF use demands rigorous hemostatic control. A tunneled catheter, combined with prophylactic rFVIII, was a viable alternative in this setting. Future advancements, such as emicizumab, may improve long-term hemostatic control and expand RRT options for this complex patient population.