ArticleJournal of clinical immunology2021
A Minimal Parameter Set Facilitating Early Decision-making in the Diagnosis of Hemophagocytic Lymphohistiocytosis.
Article in Journal of clinical immunology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 31 citations in OpenAlex.
- Development and validation of machine learning models for early diagnosis of hemophagocytic lymphohistiocytosis in pediatric Epstein-Barr virus infection.BMC medical informatics and decision making · 2026Article
- Analysis of clinical characteristics and risk factors for severe EBV infection in children-a single-center retrospective cohort study.BMC pediatrics · 2026Article
- Construction of a forest plot prediction model based on Lasso regression for Epstein-Barr virus associated hemophagocytic lymphohistiocytosis in children.Italian journal of pediatrics · 2026Article
- HIV infection complicated with talaromyces marneffei, tuberculosis, hemophagocytic lymphohistiocytosis and non-Hodgkin lymphoma: a complex case report.BMC infectious diseases · 2025Article
- Optimizing hemophagocytic lymphohistiocytosis screening in children: validation of the HLH-Screen score.Annals of hematology · 2025Article
- Multicenter validation of secondary hemophagocytic lymphohistiocytosis diagnostic criteria.Journal of internal medicine · 2025Article
- The ability of CD4Immunologic research · 2025Article
- Hemophagocytic lymphohistiocytosis as the initial manifestation of Epstein-Barr virus-related T/NK-cell lymphoproliferative disorders in a pediatric patient: a case report and literature review.Frontiers in pediatrics · 2025Article
- Pre-diagnostic trajectory of pediatric hemophagocytic lymphohistiocytosis: observations from hematological and hepatic parameters.Annals of hematology · 2024Article
- Early hematopoietic cell transplantation for familial hemophagocytic lymphohistiocytosis in a regional treatment network in Japan.International journal of hematology · 2024Article
- Allogeneic hematopoietic stem cell transplant for familial hemophagocytic lymphohistiocytosis: a case report and literature review.Frontiers in immunology · 2024Review
- Comparative study of biomarkers for the early identification of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in infectious mononucleosis.BMC infectious diseases · 2023Article
- Hemophagocytic lymphohistiocytosis secondary to virus infection and followed by lupus nephritis recurrence in a renal transplantation pediatric recipient: a case report.BMC nephrology · 2023Article
- A Three-Step Screening Procedure for Early Identification of Children at High Risk of Hemophagocytic Lymphohistiocytosis.Journal of clinical immunology · 2023Article
- Using a Minimal Parameter Set for Early Diagnosis of Hemophagocytic Lymphohistiocytosis in Non-European Children.The American journal of case reports · 2022Article
- Development of a screening score for Hemophagocytic Lymphohistiocytosis among pediatric patients with acute infection of Epstein-Barr virus.Frontiers in immunology · 2022Article
- Hemophagocytic Lymphohistiocytosis Secondary to Peripheral T Cell Lymphoma with Rapid Onset and Fatal Progression in a Young Patient: A Case Report and Review of the Literature.The American journal of case reports · 2021Review
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Authors and funding
13 authors at 8 institutions in 3 countries.
Funding
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening immune dysregulation syndrome characterized by uncontrolled immune cell activation. Timely diagnosis is important, since early treatment can improve survival rates. However, completing all assessments needed to reach ≥5 positive criteria out of the 8 HLH-2004 criteria can be time consuming and may delay timely initiation of treatment. Hence, we applied a data-driven approach to identify a minimal parameter set for early decision-making towards the initiation of HLH-specific treatment. We retrospectively evaluated 165 patients from five Dutch tertiary hospitals with suspected HLH. Sixteen pHLH (median age 0.5 years) and 70 sHLH patients (median age 8.7 years) were identified using the HLH-2004 criteria. Clustering analysis and multi-receiver operator characteristics were used to identify parameters distinctive of HLH. The presence of either increased ferritin, cytopenia in ≥2 lineages, or splenomegaly distinguished HLH from non-HLH cases with a negative predictive value of 100%. A minimal parameter set consisting of 2 major criteria (phagocytosis and splenomegaly) and 3 minor criteria (cytopenia, increased ferritin, and increased triglycerides/low fibrinogen) predicted HLH with 95% (88-99) sensitivity and 94% (86-98) specificity. This finding was replicated in an independent retrospective validation cohort of 109 US patients (n = 109). By dividing a subset of the HLH-2004 criteria into major and minor criteria, this strategy uses the evaluation of less than 5 criteria to quickly identify patients with HLH. When confirmed in a prospective setting, this approach could be of value for timely diagnosis and treatment of HLH.
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