ArticleAJNR. American journal of neuroradiology2021
MRI Patterns in Pediatric CNS Hemophagocytic Lymphohistiocytosis.
Article in AJNR. American journal of neuroradiology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed, 26 citations in OpenAlex.
- CNS-Isolated Familial Hemophagocytic Lymphohistiocytosis Masquerading as Pediatric MOGAD-ADEM: A Diagnostic Challenge.Indian journal of pediatrics · 2026Article
- Pediatric hemophagocytic lymphohistiocytosis with predominant CNS involvement: MRI features and differential diagnosis.BMC pediatrics · 2026Article
- Clinical and neuroimaging features of familial hemophagocytic lymphohistiocytosis.Pediatric radiology · 2026Article
- Unveiling pediatric secondary hemophagocytic lymphohistiocytosis: a comprehensive analysis of etiology, diagnosis, and treatment.Frontiers in immunology · 2026Article
- Prognostic scoring model incorporating clinical characteristics for pediatric hemophagocytic lymphohistiocytosis: a multicenter retrospective study.Annals of hematology · 2025Article
- Review
- Case Report: Pediatric CNS-isolated hemophagocytic lymphohistiocytosis secondary to uniparental disomy ofFrontiers in genetics · 2025Article
- Neurobrucellosis Presenting as Vocal Cord Paralysis in an Infant: Correspondence.Indian journal of pediatrics · 2024Article
- Pediatric CNS-isolated hemophagocytic lymphohistiocytosis with brain hemorrhages: a case report.BMC neurology · 2024Article
- Article
- Imaging Spectrum of Neurological Manifestations of Hemophagocytic Lymphohistiocytosis in Pediatrics: A Case Series.The Indian journal of radiology & imaging · 2023Article
- Childhood Cerebral Vasculitis : A Multidisciplinary Approach.Clinical neuroradiology · 2023Review
- Imaging of Central Nervous System Involvement in Pediatric Hematologic Disorders.Turkish archives of pediatrics · 2022Article
- Case report: Cerebellar swelling and hydrocephalus in familial hemophagocytic lymphohistiocytosis.Frontiers in pediatrics · 2022Article
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Authors and funding
15 authors at 12 institutions in 7 countries.
Funding
Abstract
background and purposeNeuroimaging has an important role in detecting CNS involvement in children with systemic or CNS isolated hemophagocytic lymphohistiocytosis. We characterized a cohort of pediatric patients with CNS hemophagocytic lymphohistiocytosis focusing on neuroradiologic features and assessed whether distinct MR imaging patterns and genotype correlations can be recognized. MATERIALS AND
methodsWe retrospectively enrolled consecutive pediatric patients diagnosed with hemophagocytic lymphohistiocytosis with CNS involvement treated at 2 pediatric neurology centers between 2010 and 2018. Clinical and MR imaging data were analyzed.
resultsFifty-seven children (40 primary, 70%) with a median age of 36 months (interquartile range, 5.5-80.8 months) were included. One hundred twenty-three MR imaging studies were assessed, and 2 broad imaging patterns were identified. Pattern 1 (significant parenchymal disease, 32/57, 56%) was seen in older children (
conclusionsDistinct MR imaging patterns correlating with clinical phenotypes and possible genetic underpinnings were recognized in this cohort of pediatric CNS hemophagocytic lymphohistiocytosis. Disruptive mutations and missense mutations with absent protein expression correlate with a younger onset age. Children with brainstem and cerebellitis patterns and a negative etiologic work-up require directed assessment for CNS hemophagocytic lymphohistiocytosis.
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