Glucose-6-phosphate Dehydrogenase Deficiency in a Male Child with Acute Liver Failure Secondary to Hemophagocytosis Lymphohistiocytosis Triggered by Enteric Fever: A Rare Tetrad
particularly in endemic regions of South Asia. 3 ALF in children may present with jaundice, coagulopathy, or encephalopathy. IntroductIonHemophagocytic lymphohistiocytosis (HLH) is a rare but severe hyperinflammatory syndrome characterized by uncontrolled immune activation, a cytokine storm, and multiorgan dysfunction.HLH, often triggered by infection, is termed secondary HLH.Familial forms of primary HLH result from genetic defects in natural killer (NK) cells and cytotoxic T cells, typically affecting perforin and intracellular vesicles.In children, HLH often manifests with prolonged fever, hepatosplenomegaly, and cytopenias and can sometimes mimic or precipitate acute liver failure (ALF).Liver involvement is common in HLH, and fulminant liver failure has been documented as a serious presenting feature, especially in neonates with familial forms, complicating diagnosis and management. 1 Infections are among the most common causes of secondary HLH.Viral agents like Epstein-Barr virus (EBV), cytomegalovirus (CMV), and influenza virus are wellknown triggers.2 Enteric fever caused by Salmonella Typhi is an unusual but reported precipitant of HLH in pediatric patients, 1-
Authors
- Indar Kumar Sharawat (ORCID: https://orcid.org/0000-0002-7003-7218)
- Sarthak Chakrabarti (ORCID: https://orcid.org/0000-0002-2552-747X)
- Juhi Tiwari (ORCID: https://orcid.org/0009-0006-0448-7241)
- Prateek Kumar Panda (ORCID: https://orcid.org/0000-0001-7831-9271)
- Anukriti Agnihotry
- Hitesh Poonia
- Ashish Jha
Institutions
- All India Institute of Medical Sciences Rishikesh (IN)
Publication Details
- Journal
- Annals of Pediatric Gastroenterology and Hepatology ISPGHAN
- Published
- 2026-10-28
- DOI
- https://doi.org/10.5005/jp-journals-11009-0197
- Primary Topic
- Autoimmune and Inflammatory Disorders Research
- Type
- article
- Field-Weighted Citation Impact
- 0.00