Hemolytic Uremic Syndrome: Recurrence after Renal Transplantation

Abstract

Hemolytic uremic syndrome (HUS) is an uncommon cause of end-stage renal failure in adults, and few data are available concerning the outcome of renal transplantation in these patients. We conducted this retrospective multicentric study to appreciate the outcome of adult renal transplant recipients whose primary disease was HUS. Sixteen patients, transplanted between 1975 and 1995, were included in the study. In each case, initial diagnosis of HUS was documented by a kidney biopsy. These 16 patients received a total of 25 allografts: 1 graft for 9 patients, 2 grafts for 5 patients, and 3 grafts for 2 patients. Nine patients (56%) developed definite clinical and pathologic evidence of recurrence on at least 1 graft. Four additional patients (25%) demonstrated only some clinical or pathologic evidence of recurrence which could not be distinguished from acute vascular rejection. Three patients had no sign of recurrence of the initial disease. The 1-year graft survival rate was 63% and the 5-year graft survival rate was 18.5%. In the group of patients with proven or possible recurrence (n = 13), the 1-year and 5-year graft survival rates were 49% and less than 10%, respectively. The recurrence was an early event, occurring before the end of the first month after transplantation in half the cases. The recurrence rate was 92% in non-nephrectomized patients and 50% in patients with bilateral nephrectomy. In the literature, 71 adult patients with primary HUS had received a total of 90 kidney grafts. Among them, 54% had a recurrence on their graft, which was diagnosed in 52% of the kidney transplants. It is note-worthy that when data from the literature are pooled with our results, the rate of recurrence appears to be significantly lower in binephrectomized patients than in patients with their native kidneys at the time of transplantation (5 of 14 versus 27 of 35 patients, respectively, p = 0.0155). By univariate analysis, no other risk factor for recurrence could be identified. Treatment with cyclosporine A did not influence the recurrence rate. We conclude that recurrence of HUS after renal transplantation is a frequent, early, and severe complication, leading rapidly to graft loss. Prospective studies are needed to confirm that bilateral nephrectomy prior to transplantation decreases the rate of recurrence.

Outcome of Renal Transplantation in Patients with Non–Shiga Toxin–Associated Hemolytic Uremic Syndrome

“…When only reports with Ͼ10 patients who had non-Stx-HUS and underwent renal transplantation are considered (6,34,36,38 -41), it emerges that more than half of patients lost the graft for HUS recurrence within the first year after transplant, despite treatment with plasma exchange and/or infusion. Cyclosporine A and FK506 administration was not associated with a higher incidence of HUS recurrence, when compared with regimens that excludes these drugs (34,38,41). Graft failure for HUS recurrence was higher in adults (60%) (34,36,38,41) than in children (20%) (34,38 -40).…”

“…Cyclosporine A and FK506 administration was not associated with a higher incidence of HUS recurrence, when compared with regimens that excludes these drugs (34,38,41). Graft failure for HUS recurrence was higher in adults (60%) (34,36,38,41) than in children (20%) (34,38 -40). The type of kidney donor, cadaveric or living related, did not modify the outcome (36,40,41).…”

“…Graft failure for HUS recurrence was higher in adults (60%) (34,36,38,41) than in children (20%) (34,38 -40). The type of kidney donor, cadaveric or living related, did not modify the outcome (36,40,41). Overall, no clinical prognostic factors that correlate with graft outcome emerge from literature.…”

45: Recurrent Atypical Hemolytic Uremic Syndrome Associated With Factor I Mutation in a Living Related Renal Transplant Recipient

“…Prior to genetic testing for aHUS, multiple studies showed recurrence of disease in the recipient together with the appearance of de novo aHUS in the donor. [20][21][22][23][24] On this basis, if a living-related donor transplantation is considered, the donor and recipient should have complete molecular testing of CFH, CFI, CD46 and CFB. In the absence of mutations in these genes or if a deletion of CFHR1 and CFHR3 is found, screening should include circulating autoantibodies for CFH and peripheral blood mononuclear cell CD46 expression.…”

The Evaluation of the Renal Transplant Candidates: Clinical Practice Guidelines

“…center study failed to find an association between the use of CsA and the rate of recurrence (346). De novo HUS/TTP can occur after exposure to CsA, and in this setting replacing CsA with tacrolimus has been reported to be beneficial in some cases (343,347,348) but not in others (349).…”