Abstract
Atypical hemolytic uremic syndrome (HUS) frequently leads to end-stage renal failure and can relapse after transplantation. A 12-year-old girl presenting with familial atypical HUS with a factor H mutation was successfully transplanted 6 years after a first transplant that had failed because of immediate recurrent HUS. Prophylactic plasma exchange before and after transplantation was used. Two months after transplantation, concomitant with a reduction in plasma exchange frequency, the plasma creatinine increased from 70 µmol/l to 194 µmol/l in 2 weeks without thrombocytopenia or signs of hemolytic anemia. The patient had minimal clinical symptoms and a presumptive diagnosis of graft rejection was made. Despite treatment with six daily pulses of methylprednisolone, plasma creatinine continued to increase and a graft biopsy was therefore undertaken. This showed the typical appearance of a thrombotic microangiopathy without any evidence of rejection. Despite daily plasmapheresis and replacement of cyclosporine with tacrolimus, there was no improvement and transplant nephrectomy was undertaken. This patient demonstrates that HUS can recur in a kidney transplant without the diagnostic hematological features and emphasizes the need for early transplant biopsy in such patients showing a decline in transplant function.
References
Richards A, Buddles MR, Donne RL, Kaplan BS, Kirk E, Venning MC, Tielemans CL, Goodship JA, Goodship THJ (2001) Factor H mutations in haemolytic uremic syndrome cluster in exons 18-20, a domain important for host cell recognition. Am J Hum Genet 68:485–490
Ruggenenti P (2002) Post-transplant haemolytic uremic syndrome. Kidney Int 62:1093–1104
Loirat C, Niaudet P (2003) The risk of recurrence of haemolytic uremic syndrome after renal transplantation in children. Pediatr Nephrol 18:1095–1101
Neumann HP, Salzmann M, Bohnert-Iwan B, Mannuelian T, Skerka Lenk D, Bender BU, Cybulla M, Riegler P, Konigsrainer A, Neyer U, Bock A, Widmer U, Male DA, Franke G, Zipfel PF (2003) Haemolytic uraemic syndrome and mutations of the factor H gene: a registry-based study of German speaking countries. J Med Genet 40:676–681
Davin JC, Gruppen M, Bouts AHM, Groothoff JW, Ploos van Amstel S, Surachno J, Berge IJM ten, Weening JJ (1999) Relapse of atypical haemolytic uremic syndrome after kidney transplantation: role of ATG and failure of mycophenolate mofetil as rescue therapy. Nephrol Dial Transplant 14:984–987
Abraham KA, Little MA, Dorman AM, Walshe JJ (2000) Hemolytic-uraemic syndrome in association with both cyclosporine and tacrolimus. Transpl Int 13:443–447
Ducloux D, Rebibou JM, Semhoun Ducloux S (1998) Recurrence of haemolytic-uremic syndrome in renal transplant recipients—a meta-analysis. Transplantation 65:1405–1407
Quan A, Sullivan E, Alexander S (2001) Recurrence of HUS after renal transplantation in children. Transplantation 72:742–745
Nathanson S, Frémeaux-Bacchi V, Deschene G (2001) Successful therapy in the haemolytic uremic syndrome with factor H deficiency. Pediatr Nephrol 16:554–556
Hebert D, Sibley RK, Mauer SM (1986) Recurrence of haemolytic uremic syndrome in renal transplant recipients. Kidney Int 30:S51–S52
Magen D, Oliven A, Shechter Y, Elhasid R, Bar-Joseph G, Zelikovic I (2001) Plasmapheresis in a very young infant with atypical haemolytic uremic syndrome. Pediatr Nephrol 16:87–90
Robson W, Leung A (1991) The successful treatment of atypical HUS with plasmapheresis. Clin Nephrol 35:119–122
Loirat C, Sonsino E, Hinglais N, Jais JP, Landais P, Fermanian J (1988) Treatment of the childhood hemolytic uremic syndrome with plasma. Pediatr Nephrol 2:279–285
Deschênes G, Veyradier A, Cloarec S, Benoit S, Desbois I, Gruel Y, Nivet H (2002) Plasma therapy in von Willebrand factor protease deficiency. Pediatr Nephrol 17:867–870
Remuzzi G, Ruggenenti P, Codazzi D, Noris M, Caprioli J, Locatelli G, Gridelli B (2002 ). Combined kidney and liver transplantation for familial haemolytic uraemic syndrome. Lancet 359:1671–1672
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Olie, K.H., Florquin, S., Groothoff, J.W. et al. Atypical relapse of hemolytic uremic syndrome after transplantation. Pediatr Nephrol 19, 1173–1176 (2004). https://doi.org/10.1007/s00467-004-1565-2
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DOI: https://doi.org/10.1007/s00467-004-1565-2