ArticleChinese medical journal2018
Central Nervous System Involvement in Hemophagocytic Lymphohistiocytosis in Adults: A Retrospective Analysis of 96 Patients in a Single Center.
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 26 papers, 3 of them syntheses that pooled it.
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Who cites it
26 citing papers in PubMed, 3 syntheses or guidelines pooled it, 42 citations in OpenAlex.
- Enteric Fever-Associated Hemophagocytic Lymphohistiocytosis: A Systematic Review of Clinical Characteristics and Outcomes.The American journal of tropical medicine and hygiene · 2024Pooled 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
- 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
- Neurological Involvement in Adult-Onset Secondary Hemophagocytic Lymphohistiocytosis: Clinical Features and Prognostic Implications.Brain and behavior · 2026Article
- Clinical characteristics and prognostic factors in adult macrophage activation syndrome: an observational study in a Chinese cohort.Lupus science & medicine · 2025Observational
- Prognostic scoring model incorporating clinical characteristics for pediatric hemophagocytic lymphohistiocytosis: a multicenter retrospective study.Annals of hematology · 2025Article
- Hemophagocytic Lymphohistiocytosis (HLH) in Patients with Tick-Borne Illness: A Scoping Review of 98 Cases.Infectious disease reports · 2024Article
- Brain MRI imaging markers associated with death in children with central nervous system involvement of hemophagocytic lymphohistiocytosis.European radiology · 2024Article
- Probable secondary hemophagocytic lymphohistiocytosis manifesting as central nervous system lesions after COVID-19 vaccination: a case report.Frontiers in neurology · 2024Article
- 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
- Case report: Hemophagocytic lymphohistiocytosis complicated by multiple organ dysfunction syndrome following aseptic encephalitis.Frontiers in immunology · 2023Article
- Review
- An Elusive Seizure.The Neurohospitalist · 2022Article
- Central Nervous System Hemophagocytic Lymphohistiocytosis (CNS-HLH) from Leptomeningeal Anaplastic Large Cell Lymphoma : Mild Clinical Neurologic Syndrome with Extensive Multifocal White Matter Disease.Clinical neuroradiology · 2021Article
- Prominent hemophagocytosis in cerebrospinal fluid in a patient with pneumococcal meningitis.Clinical case reports · 2021Article
- Brain dysfunction in COVID-19 and CAR-T therapy: cytokine storm-associated encephalopathy.Annals of clinical and translational neurology · 2021Review
- Case Report: Meningoencephalitis With Thrombotic Occlusive Vasculopathy in a Young EBV-Naïve Boy Is Associated With a NovelFrontiers in immunology · 2021Article
- Clinical Characteristics of Adult Hemophagocytic Lymphohistiocytosis in the Emergency Department.International journal of general medicine · 2021Article
- An Atypical Presentation of Hemophagocytic Lymphohistiocytosis (HLH) Secondary to Occult Hodgkin Lymphoma.Case reports in hematology · 2021Article
- New-onset non-convulsive status epilepticus in an adult with hemophagocytic lymphohistiocytosis: a case report.Quantitative imaging in medicine and surgery · 2020Article
Corrections and comments
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Authors and funding
5 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundHemophagocytic lymphohistiocytosis (HLH) is a life-threatening clinical syndrome. Central nervous system (CNS) involvement is a severe complication, which can lead to rapid disease development and higher morality. However, this has not been given enough attention in adult HLH. Therefore, we carried out this study to analyze the clinical features, laboratory findings, treatment outcomes, and other characteristics of adult HLH with CNS involvement.
methodsA retrospective analysis of 96 adult patients with HLH combined with CNS involvement between June 2003 and December 2016 was conducted. Clinical features, cerebrospinal fluid (CSF) features, image changes, and therapeutic outcomes were analyzed.
resultsAmong the 96 patients, 86 had various CNS symptoms and 33 (38.4%) had already presented symptoms before the HLH diagnosis was confirmed. A total of 59 patients received CSF examinations and showed abnormalities in 23 patients (39.0%). Seventy patients received imaging examinations and the results showed fifty patients with imaging changes (71.4%). Fifty-seven patients received multiple rounds of repeated intrathecal injection therapy and 35 patients improved (61.4%). As for the multiple analyses of effective factors on survival time, the results showed that the effects of combined Epstein-Barr virus (EBV) infection (P = 0.026, Exp(B) = 2.309, 95% confidence interval [CI] [1.108, 4.823) and intrathecal injection therapy (P = 0.013, Exp(B) = 0.422, 95% CI [0.214, 0.831]) on the survival time of the CNS-HLH patients were significant.
conclusionsComplication with EBV infection is a risk factor, and intrathecal injection is a protective factor. CNS involvement in HLH is not rare, which can result in a poor prognosis. Multiple rounds of repeated intrathecal injection therapy can improve the prognosis of CNS-HLH patients.
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