Adult Post–Kidney Transplant Familial Atypical Hemolytic Uremic Syndrome Successfully Treated With Eculizumab: A Case Report and Literature Review

Abstract

Hemolytic uremic syndrome is the triad of nonimmune microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. When not associated with enteric infection, it is classified as atypical hemolytic uremic syndrome (aHUS) and carries a worse outcome with high mortality rate and up to 50% of the survivors will end up with end-stage renal disease. Renal transplant was restricted to a very small percentage of patients due to high recurrence rate posttransplant that approaches 90%. Our case describes a posttransplant adult patient with familial aHUS whom was successfully treated with eculizumab. We also reviewed all other reported cases of adult posttransplant aHUS, both familial and sporadic, which were treated with eculizumab. In summary, eculizumab might expand the utility of renal transplant for patients with end-stage renal disease due to aHUS.

Eculizumab in atypical hemolytic uremic syndrome: strategies toward restrictive use

“…In all of them, eculizumab therapy had been suboptimal (late initiation of therapy or too early discontinuation) and/or renal function had been compromised before the onset of aHUS. We identified ten patients, with at least four patients [76, 87–89] at high risk of recurrence [15], in whom eculizumab was initiated within 28 days after onset of aHUS. Early treatment resulted in full recovery of renal function: median (range) serum creatinine was 113 μmol l −1 (53–159) before aHUS recurrence and 116 μmol l −1 (53–180) after treatment of recurrence [76, 87, 88, 90–95].…”