Paroxysmal nocturnal hemoglobinuria masquerading as hemolytic uremic syndrome: a Case Report

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disorder of intravascular hemolysis caused by a somatic mutation in the gene responsible for glycosylphosphatidylinositol (GPI)-anchored complement regulatory proteins. This mutation leads to the production of abnormal blood cell clones la...

Full description

Saved in:
Bibliographic Details
Main Authors: Menghan Gao, Bo Liu, Jianping Yao, Fuhan Huang
Format: Article
Language:English
Published: Frontiers Media S.A. 2025-07-01
Series:Frontiers in Medicine
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fmed.2025.1553168/full
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1850074898646433792
author Menghan Gao
Bo Liu
Jianping Yao
Fuhan Huang
author_facet Menghan Gao
Bo Liu
Jianping Yao
Fuhan Huang
author_sort Menghan Gao
collection DOAJ
description Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disorder of intravascular hemolysis caused by a somatic mutation in the gene responsible for glycosylphosphatidylinositol (GPI)-anchored complement regulatory proteins. This mutation leads to the production of abnormal blood cell clones lacking CD55 and CD59. PNH can result in renal damage. The challenge of early identification and diagnosis leads to misdiagnosis as other intravascular hemolytic conditions. This paper presents a case that began with fever, diarrhea, and acute renal failure, initially misdiagnosed as atypical hemolytic uremic syndrome (aHUS) but later confirmed as PNH through renal biopsy and related diagnostic tests. After treatment, the patient’s renal function recovered, and anemia improved. Intravascular hemolysis is a prominent feature common to both PNH and HUS. They exhibit similar clinical manifestations, which pose a challenge for differential diagnosis. Unlike previous reports, the patient in this case denied any history of hematologic disorders, which made the diagnosis more challenging.
format Article
id doaj-art-221d50081c2e4267bb4fcb3e40128c20
institution DOAJ
issn 2296-858X
language English
publishDate 2025-07-01
publisher Frontiers Media S.A.
record_format Article
series Frontiers in Medicine
spelling doaj-art-221d50081c2e4267bb4fcb3e40128c202025-08-20T02:46:28ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2025-07-011210.3389/fmed.2025.15531681553168Paroxysmal nocturnal hemoglobinuria masquerading as hemolytic uremic syndrome: a Case ReportMenghan Gao0Bo Liu1Jianping Yao2Fuhan Huang3Department of Nephrology, Huzhou Central Hospital, Fifth School of Clinical Medicine of Zhejiang Chinese Medical University, Affiliated Central Hospital of Huzhou University, Huzhou, Zhejiang, ChinaDepartment of Nephrology, Huzhou Central Hospital, Fifth School of Clinical Medicine of Zhejiang Chinese Medical University, Affiliated Central Hospital of Huzhou University, Huzhou, Zhejiang, ChinaDepartment of Endocrinology, Huzhou Central Hospital, Fifth School of Clinical Medicine of Zhejiang Chinese Medical University, Affiliated Central Hospital of Huzhou University, Huzhou, Zhejiang, ChinaDepartment of Nephrology, Huzhou Central Hospital, Fifth School of Clinical Medicine of Zhejiang Chinese Medical University, Affiliated Central Hospital of Huzhou University, Huzhou, Zhejiang, ChinaParoxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disorder of intravascular hemolysis caused by a somatic mutation in the gene responsible for glycosylphosphatidylinositol (GPI)-anchored complement regulatory proteins. This mutation leads to the production of abnormal blood cell clones lacking CD55 and CD59. PNH can result in renal damage. The challenge of early identification and diagnosis leads to misdiagnosis as other intravascular hemolytic conditions. This paper presents a case that began with fever, diarrhea, and acute renal failure, initially misdiagnosed as atypical hemolytic uremic syndrome (aHUS) but later confirmed as PNH through renal biopsy and related diagnostic tests. After treatment, the patient’s renal function recovered, and anemia improved. Intravascular hemolysis is a prominent feature common to both PNH and HUS. They exhibit similar clinical manifestations, which pose a challenge for differential diagnosis. Unlike previous reports, the patient in this case denied any history of hematologic disorders, which made the diagnosis more challenging.https://www.frontiersin.org/articles/10.3389/fmed.2025.1553168/fullacute kidney injurycase reporthemolytic uremic syndromehemosiderin depositionmisdiagnosisparoxysmal nocturnal hemoglobinuria
spellingShingle Menghan Gao
Bo Liu
Jianping Yao
Fuhan Huang
Paroxysmal nocturnal hemoglobinuria masquerading as hemolytic uremic syndrome: a Case Report
Frontiers in Medicine
acute kidney injury
case report
hemolytic uremic syndrome
hemosiderin deposition
misdiagnosis
paroxysmal nocturnal hemoglobinuria
title Paroxysmal nocturnal hemoglobinuria masquerading as hemolytic uremic syndrome: a Case Report
title_full Paroxysmal nocturnal hemoglobinuria masquerading as hemolytic uremic syndrome: a Case Report
title_fullStr Paroxysmal nocturnal hemoglobinuria masquerading as hemolytic uremic syndrome: a Case Report
title_full_unstemmed Paroxysmal nocturnal hemoglobinuria masquerading as hemolytic uremic syndrome: a Case Report
title_short Paroxysmal nocturnal hemoglobinuria masquerading as hemolytic uremic syndrome: a Case Report
title_sort paroxysmal nocturnal hemoglobinuria masquerading as hemolytic uremic syndrome a case report
topic acute kidney injury
case report
hemolytic uremic syndrome
hemosiderin deposition
misdiagnosis
paroxysmal nocturnal hemoglobinuria
url https://www.frontiersin.org/articles/10.3389/fmed.2025.1553168/full
work_keys_str_mv AT menghangao paroxysmalnocturnalhemoglobinuriamasqueradingashemolyticuremicsyndromeacasereport
AT boliu paroxysmalnocturnalhemoglobinuriamasqueradingashemolyticuremicsyndromeacasereport
AT jianpingyao paroxysmalnocturnalhemoglobinuriamasqueradingashemolyticuremicsyndromeacasereport
AT fuhanhuang paroxysmalnocturnalhemoglobinuriamasqueradingashemolyticuremicsyndromeacasereport