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Hemophilia A is a congenital deficiency of blood coagulation factor VIII (fVIII) that causes prolonged bleeding time. Dialysis in these patients can be challenging and several aspects must be considered, namely the patient’s will and characteristics, the dialysis modality, the dialysis access, hypocoagulation and the need for fVIII infusion.
We present two cases of patients undergoing hemodialysis with hemophilia A and compare them with the available literature.
The first case is of a 53-year-old male diagnosed with moderate hemophilia A (fVIII 3%), treated with fVIII on demand, and with autosomal dominant polycystic kidney disease (ADKP). Respecting the patient's wishes, hemodialysis was considered and a left radialcephalic arteriovenous fistula (AVF) was created, after prophylactic administration of fVIII. He started hemodialysis using the AVF, without hypocoagulation, initially single needle and later with double needle and post-dilution hemodiafiltration. No circuit clotting or bleeding complications associated with cannulation were documented after 5 months of follow-up.
The second case is of 64-years-old male with sever hemophilia A (fVIII < 1%), treated with prophylactic fVIII administration thrice a week and end stage renal disease of unknow etiology. A radiochephalic AVF was placed with prophylactic fVIII administration. He started hemodialysis with the same prescription as the first case, yet fVIII administration was shifted to a post dialysis schedule. There were no significant bleeding or clotting episodes during the follow-up years before the patient was transferred to a different hemodialysis facility.
To our knowledge, there are only case reports and small case series on this subject, with varied hemophilia A severity and hemodialysis approaches. Although some authors defend the use of tunneled central venous catheter as the definite vascular access, there are case reports of successful hemodialysis using AVF and arteriovenous graft and we believe that given the best practices, young age of the patients and the overall risk reduction, AVF should be the first vascular access option. With the appropriate caution, both cases demonstrate that it’s a safe option.
In terms of hypocoagulation, there are several approaches in the literature. Given the tendency for prolonged bleeding time of hemophilic patients and the absence of clear hypocoagulation strategies, we favor obliviating additional hypocoagulation. In both cases, no additional fVIII was administered because of the technique, although days of administration were adapted to fit dialysis’ days. In our patients, hemostasis time was less than 10 minutes, suggesting that the reported supplementation of fVIII in each session may not be needed.
The prevalence of chronic kidney disease in patients with Hemophilia A is increasing, making it important to the Nephrologist to be aware of this condition and giving rise to the need for further studies to determine the best dialysis practices for these patients.
In our experience, it was possible to have the best vascular access, the most efficient hemodialysis modality, with optimal volumes and dialysis’ dose, and not to use hypocoagulation or administer additional fVIII, without clinical or technical complications.