ArticleChinese medical journal2018
Central Nervous System Involvement in 179 Chinese Children with Hemophagocytic Lymphohistiocytosis.
Article in Chinese medical journal, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers, 2 of them syntheses that pooled it.
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Who cites it
18 citing papers in PubMed, 2 syntheses or guidelines pooled it, 38 citations in OpenAlex.
- The 2022 EULAR/ACR Points to Consider at the Early Stages of Diagnosis and Management of Suspected Haemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome (HLH/MAS).Arthritis & rheumatology (Hoboken, N.J.) · 2023Pooled it
- The 2022 EULAR/ACR points to consider at the early stages of diagnosis and management of suspected haemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS).Annals of the rheumatic diseases · 2023Pooled it
- Early management of hemophagocytic lymphohistiocytosis/macrophage activation syndrome: a systematic literature review.EULAR rheumatology open · 2026Review
- Neurological Involvement in Adult-Onset Secondary Hemophagocytic Lymphohistiocytosis: Clinical Features and Prognostic Implications.Brain and behavior · 2026Article
- Unveiling pediatric secondary hemophagocytic lymphohistiocytosis: a comprehensive analysis of etiology, diagnosis, and treatment.Frontiers in immunology · 2026Article
- Case Report: Munc13-4 deficiency presenting with autoimmune neuropathy years before FLH: implications for early genetic screening and HSCT timing.Frontiers in immunology · 2026Article
- Lumbar Puncture Knowledge and Attitudes among Patients, Caregivers, and Healthcare Workers in Rural Zambia.The American journal of tropical medicine and hygiene · 2025Article
- Development of an early mortality risk prediction model for pediatric patients with secondary hemophagocytic lymphohistiocytosis.Italian journal of pediatrics · 2025Article
- Clinical characteristics and prognostic analysis of pediatric hemophagocytic lymphohistiocytosis using lasso-logistic regression.Annals of hematology · 2024Article
- Brain MRI imaging markers associated with death in children with central nervous system involvement of hemophagocytic lymphohistiocytosis.European radiology · 2024Article
- [Systemic lupus erythematosus associated macrophage activation syndrome with neuropsychiatric symptoms: A report of 2 cases].Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences · 2023Article
- Central nervous system involvement in adult-onset hemophagocytic lymphohistiocytosis secondary to lymphoma: a case presentation and literature analysis.Quantitative imaging in medicine and surgery · 2023Article
- Central nervous system involvement and thrombocytopenia as predictors of mortality in children with hemophagocytic lymphohistiocytosis.Frontiers in pediatrics · 2022Article
- Successful rescue of a lethal Griscelli syndrome type 2 presenting with neurological involvement and hemophagocytic lymphohistiocytosis: a case report.BMC pediatrics · 2021Article
- Treatment of pediatric primary hemophagocytic lymphohistiocytosis with the HLH-94/2004 regimens and hematopoietic stem cell transplantation in China.Annals of hematology · 2020Article
- [Treatment experience of hemophagocytic lymphohistiocytosis with severe central nervous system involvement in a child].Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics · 2020Article
- Neurological Abnormality Could be the First and Only Symptom of Familial Hemophagocytic Lymphohistiocytosis: Report of Two Families.Chinese medical journal · 2018Article
- Current Treatment for Central Nervous System Involvement in Children with Hemophagocytic Lymphohistiocytosis.Chinese medical journal · 2018Article
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Authors and funding
10 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundCentral nervous system (CNS) involvement is found in many patients with hemophagocytic lymphohistiocytosis (HLH). In this study, we mainly analyzed neurological symptoms, imaging findings, cerebrospinal fluid (CSF), and their relationship with outcomes of HLH children.
methodsRelated data of 179 Chinese pediatric patients with HLH admitted to our center from January 2010 to December 2015 were analyzed retrospectively. Diagnosis and treatment were based on the HLH-2004 protocol. Two-tailed Chi-squared test was used to compare between different groups, and Kaplan-Meier survival curves were used to analyze the overall survival (OS) of patients with HLH.
resultsIn the present study, 21.2% (38/179) of total patients had neurological symptoms including seizure, irritability, somnolence, and unconsciousness. There were 80 (50.0%, excluding 19 patients without imaging data) patients with cranial imaging abnormalities. There were 14.7% (17/116, excluding 63 patients who did not accept lumbar puncture) of patients with abnormal CSF results. CNS involvement is defined as abnormalities in one or more of CNS symptoms, radiological findings, and CSF. Thus, 60.3% of them had CNS involvement. As for the prognosis, the median follow-up time was 3.2 years (17 lost to follow-up). The probable 3-year OS of children was higher without CNS involvement (86.0% ± 4.6%) than those with CNS involvement (68.9% ± 4.9%, hazard ratio [HR] = 2.286, P = 0.019). Among them, the probable 3-year OS of children without CNS symptoms was 76.0% ± 3.8%, higher than with CNS symptoms (59.5% ± 8.1%, HR = 2.147, P = 0.047). The 3-year OS of children with abnormal CSF was 64.7% ± 11.6%, compared with normal CSF (85.1% ± 3.7%, HR = 0.255, P = 0.038).
conclusionsHLH patients with CNS involvement might have worse outcomes compared with those without CNS involvement, and CNS symptoms and CSF changes are more important to access the prognosis than imaging abnormality.
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