Recurrence of hemolytic uremic syndrome after renal transplantation.
Non-Shiga toxin-associated hemolytic uremic syndrome (non-Stx-HUS) is a rare disease. The clinical outcome is often unfavorable: 50% of patients progress to end-stage renal failure. Several mutations in complement regulatory genes predispose to non-Stx-HUS. Transplantation outcomes are poor among patients with either mutation in the genes encoding complement H or I factors, with 80% graft loss due to HUS recurrence. In contrast, patients with mutation in the gene encoding MCP have no disease relapse after transplantation. There are no treatment guidelines for non-Stx-HUS recurrence. Herein we have presented 8 patients with non-Stx-HUS recurrence after transplantation during the last 10 years in the South of France. HUS recurrence, which occurred early after transplantation in all but 1 patient, was treated by plasma exchange (PE) with substitution by fresh frozen plasma (FFP). Three patients still treated with long-term plasma therapy have no recurrence at 15, 19, or 24 months. An international registry would help to define new guidelines.
Seitz
B
B
Hôpital Pasteur, 30 Avenue de la Voie Romaine, 06002 Nice Cedex 1, France. barbara.seitz@wanadoo.fr
Albano
L
L
Vocila
F
F
Mzoughi
S
S
Aoudia
R
R
Guitard
J
J
Ribes
D
D
Vachet-Copponat
H
H
Mourad
G
G
Bienaimé
F
F
Dahan
P
P
Frémeaux-Bacchi
V
V
Cassuto
E
E
D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000235:genetics / Q000601:surgery*; D006801:Humans; D007676:Kidney Failure, Chronic / Q000601:surgery*; D016030:Kidney Transplantation / Q000009:adverse effects*; D008297:Male; D009154:Mutation; D012008:Recurrence; D012086:Reoperation; D012189:Retrospective Studies