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. 2024 Apr 24;5(3):548-553.
doi: 10.1002/jha2.902. eCollection 2024 Jun.

Eculizumab for Shiga-toxin-induced hemolytic uremic syndrome in adults with neurological involvement

Benjamin J Lee  1   2 , Zhaohui Arter  3 , Jean Doh  1 , Shawn P Griffin  1   2 , Pongthep Vittayawacharin  3   4 , Steven Atallah  1   2 , Kevin R Shieh  3 , Minh-Ha Tran  5 , Sonata Jodele  6   7 , Piyanuch Kongtim  3 , Stefan O Ciurea  3
Affiliations

Eculizumab for Shiga-toxin-induced hemolytic uremic syndrome in adults with neurological involvement

Benjamin J Lee et al. EJHaem. .

Abstract

The role of eculizumab in treating Shiga-toxin-producing Escherichia coli (STEC) hemolytic uremic syndrome (HUS) patients with neurological involvement remains unclear. We describe two distinctly different STEC-HUS patients with neurologic involvement successfully managed with eculizumab, and perform a literature review of all published cases. Both patients had complete resolution of neurological symptoms after initiation of eculizumab. Eighty patients with STEC-HUS treated with eculizumab were identified in the literature, 68.7% had complete resolution of neurological symptoms. Based on our experience and literature review, three prevailing themes were noted: 1) Early eculizumab administration optimized neurological outcomes, 2) Symptom resolution may not be immediate, neurological symptoms may initially worsen before improvement, and 3) Plasma exchange yielded no benefit. Early administration of eculizumab may reverse neurotoxicity in patients with STEC-HUS.

Keywords: STEC‐HUS; Shiga‐toxin producing Escherichia coli; eculizumab; hemolytic uremic syndrome; neurological involvement.

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Conflict of interest statement

Sonata Jodele has received consulting fees, payments, or honoraria from Omeros, Sobi, and Alexion Pharmaceuticals. Sonata Jodele has also received grant funding from Alexion Pharmaceuticals. The remaining authors declare no conflict of interest.

Figures

FIGURE 1
FIGURE 1
Temporal association for Case #1 between eculizumab dosing with (A) total complement (CH50) and eculizumab level, (B) CH50 and terminal complement (sC5b‐9), (C) haptoglobin, and (D) packed red blood cell transfusion requirement. ECU, eculizumab; PLEX, plasma exchange; pRBC, packed red blood cell.
See this image and copyright information in PMC

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