Eculizumab and Belatacept for De Novo Atypical Hemolytic Uremic Syndrome Associated With CFHR3-CFHR1 Deletion in a Kidney Transplant Recipient: A Case Report

• DOI: 10.1016/j.transproceed.2016.11.008
• Publication Date: 2017-01-16T07:51:23Z
• Authors: P. Dedhia; A. Govil; G. Mogilishetty; R.R. Alloway; E.S. Woodle; B.G. Abu Jawdeh
• Keywords: Adult; Graft Rejection; Heterozygote; Blood Proteins; Antibodies, Monoclonal, Humanized; Kidney Transplantation; Tacrolimus; 3. Good health; Abatacept; 03 medical and health sciences; Complement Inactivating Agents; Postoperative Complications; 0302 clinical medicine; Complement C3b Inactivator Proteins; Humans; Female; Immunosuppressive Agents; Atypical Hemolytic Uremic Syndrome

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.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.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.