P Dedhia1, A Govil2, G Mogilishetty2, R R Alloway2, E S Woodle3, B G Abu Jawdeh2. 1. Division of Nephrology and Hypertension, University of Cincinnati, Cincinnati, Ohio, United States. Electronic address: drparasdedhia@gmail.com. 2. Division of Nephrology and Hypertension, University of Cincinnati, Cincinnati, Ohio, United States. 3. Division of Transplant Surgery, University of Cincinnati, Cincinnati, Ohio, United States.
Abstract
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is associated with significant morbidity and mortality and occurs due to genetic or acquired abnormalities that result in the dysregulation of the alternative complement pathway. CASE REPORT: We report a case of post-living kidney transplantation de novo aHUS in a setting of heterozygous deletion in the complement factor H-related protein (CFHR)3-CFHR1 gene. The aHUS episode was possibly triggered by antibody-mediated rejection or tacrolimus. The patient responded well to eculizumab and substituting belatacept for tacrolimus. Her serum creatinine level was stable at 1.5 mg/dL after 2.5 years of follow-up. CONCLUSION: This case highlights the success of using a strategy that combines eculizumab and belatacept, as an alternative to calcineurin inhibitors, in treating aHUS in a patient with heterozygous deletion in the CFHR3-CFHR1 gene.
BACKGROUND:Atypical hemolytic uremic syndrome (aHUS) is associated with significant morbidity and mortality and occurs due to genetic or acquired abnormalities that result in the dysregulation of the alternative complement pathway. CASE REPORT: We report a case of post-living kidney transplantation de novo aHUS in a setting of heterozygous deletion in the complement factor H-related protein (CFHR)3-CFHR1 gene. The aHUS episode was possibly triggered by antibody-mediated rejection or tacrolimus. The patient responded well to eculizumab and substituting belatacept for tacrolimus. Her serum creatinine level was stable at 1.5 mg/dL after 2.5 years of follow-up. CONCLUSION: This case highlights the success of using a strategy that combines eculizumab and belatacept, as an alternative to calcineurin inhibitors, in treating aHUS in a patient with heterozygous deletion in the CFHR3-CFHR1 gene.
Authors: Johannes Münch; Anette Bachmann; Maik Grohmann; Christof Mayer; Michael Kirschfink; Tom H Lindner; Carsten Bergmann; Jan Halbritter Journal: Clin Kidney J Date: 2017-07-10