Atypical relapse of hemolytic uremic syndrome after transplantation
Source: Pediatric Nephrology, Volume 19, Number 10, October 2004 , pp. 1173-1176(4)
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.
Document Type: Research Article
Affiliations: 1: Pediatric Nephrology Unit, Emma Children’s Hospital, Academic Medical Center, Amsterdam, The Netherlands, 2: Pathology Department, Academic Medical Center, Amsterdam, The Netherlands, 3: Institute of Human Genetics, University of Newcastle upon Tyne, Newcastle upon Tyne, UK, 4: Pediatric Nephrology Unit, Emma Children’s Hospital, Academic Medical Center, Amsterdam, The Netherlands, Email: firstname.lastname@example.org
Publication date: October 2004