A Clinical Case of Atypical Hemolytic Uremic Syndrome in Remission: A Step Towards Personalized Treatment

Atypical hemolytic uremic syndrome (aHUS) is a rare, complement‑mediated thrombotic microangiopathy requiring early recognition and treatment. We present the case of a 57‑year‑old woman with aHUS triggered by a diarrheal illness, who developed acute kidney injury and hematological abnormalities. Eculizumab was initiated within 48 hours, alongside plasma exchange. The patient achieved hematologic remission within two weeks and recovered renal function within one month. Genetic testing revealed no pathogenic variants in complement‑related genes. Based on clinical stability and genetic findings, eculizumab was discontinued after six months. At the 12‑month follow‑up, the patient remained in remission with preserved renal function. This case supports the safety of eculizumab withdrawal in genetically negative aHUS under close monitoring.

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Publication Details

Journal
Portuguese Kidney Journal (PKJ)
Published
2025-08-16
DOI
https://doi.org/10.71749/pkj.100
Primary Topic
Complement system in diseases
Type
article
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article

A Clinical Case of Atypical Hemolytic Uremic Syndrome in Remission: A Step Towards Personalized Treatment

Portuguese Kidney Journal (PKJ)
Complement system in diseases
article

A Clinical Case of Atypical Hemolytic Uremic Syndrome in Remission: A Step Towards Personalized Treatment

article en

Abstract

Atypical hemolytic uremic syndrome (aHUS) is a rare, complement‑mediated thrombotic microangiopathy requiring early recognition and treatment. We present the case of a 57‑year‑old woman with aHUS triggered by a diarrheal illness, who developed acute kidney injury and hematological abnormalities. Eculizumab was initiated within 48 hours, alongside plasma exchange. The patient achieved hematologic remission within two weeks and recovered renal function within one month. Genetic testing revealed no pathogenic variants in complement‑related genes. Based on clinical stability and genetic findings, eculizumab was discontinued after six months. At the 12‑month follow‑up, the patient remained in remission with preserved renal function. This case supports the safety of eculizumab withdrawal in genetically negative aHUS under close monitoring.

Portuguese Kidney Journal (PKJ)
Universidade Politécnica de Castelo Branco (PT)
Good health and well-being
Openalex Percentile: Top 41%
Complement system in diseases
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