ArticleJournal of internal medicine2025
Multicenter validation of secondary hemophagocytic lymphohistiocytosis diagnostic criteria.
Article in Journal of internal medicine, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed.
- Machine learning for prediction in secondary hemophagocytic lymphohistiocytosis: real progress, real limits, and the potential of synthetic data.Intensive care medicine · 2026Article
- Hemophagocytic lymphohistiocytosis in critically ill patients: A guide for clinicians.World journal of critical care medicine · 2026Review
- Article
- Hyperferritinemia: Pathophysiology, Etiologies, and Diagnostic Approach.European journal of haematology · 2026Review
- Article
- Development and external validation of a machine learning prediction model for Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children using routine blood parameters: a retrospective cohort study.BMC infectious diseases · 2026Article
- Article
- Hemopoietic stem cell transplantation for infectious mononucleosis-related aplastic anemia.Annals of hematology · 2026Article
- Secondary Hemophagocytic Lymphohistiocytosis in a Rocky Mountain Spotted Fever Patient.Cureus · 2026Article
- CXCL9 as a novel prognostic marker to identify high-risk adults with hemophagocytic lymphohistiocytosis.Blood · 2026Article
- The Paucity of Typical Pathology: An Autopsy Series of Typhus Group Rickettsiosis-Associated Hemophagocytic Lymphohistiocytosis.Pathogens (Basel, Switzerland) · 2026Article
- Early Prediction Model for Etoposide-Based Protocols Resistance in Pediatric HLH.Pediatric health, medicine and therapeutics · 2026Article
- Hemophagocytic Syndromes in Adults: Real-World Data on Mortality from a Tertiary Reference Center.Acta haematologica · 2026Article
- Histiocyte Society blueprint for hemophagocytic lymphohistiocytosis research: deciphering underlying disease mechanisms to optimize diagnosis and therapy.Haematologica · 2025Review
- Article
- Clinical and Laboratory Features of Hemophagocytic Lymphohistiocytosis in People With Disseminated Histoplasmosis.Open forum infectious diseases · 2025Article
- Secondary Hemophagocytic Lymphocytosis in Inflammatory Bowel Disease.Hematology reports · 2025Article
- Severe hemophagocytic lymphohistiocytosis secondary to disseminated histoplasmosis in a patient with HIV/AIDS: a case report.Annals of medicine and surgery (2012) · 2025Article
- Multicenter validation of secondary hemophagocytic lymphohistiocytosis diagnostic criteria.Journal of internal medicine · 2025Article
- Primary prostatic Burkitt's lymphoma complicated with hemophagocytic lymphohistiocytosis: a case report and literature review.Frontiers in oncology · 2025Article
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Authors and funding
24 authors.
Funding
Abstract
backgroundFive fulfilled hemophagocytic lymphohistiocytosis (HLH)-2004 criteria, and the HScore are widely used and recommended by international expert consensus to diagnose secondary HLH. Both diagnostic scores have never been validated in heterogeneous patient cohorts of secondary HLH patients. We aimed to systematically optimize and validate diagnostic criteria of secondary HLH using a multicenter approach.
methodsWe developed optimized criteria in our cohort of critically ill patients as a first step. We next validated these new criteria together with the original and modified HLH-2004 criteria as well as the HScore using original data of 13 published cohorts, which were identified by a systematic literature search.
resultsThe best performing HLH diagnostic criteria sets over all 13 validation cohorts were the original HLH-2004 criteria with a decreased cut-off (cut-off 4, mean sensitivity 86.5%, mean specificity 86.1%), followed by the revised HLH-2004 criteria (natural killer cell activity removed; cut-off 4, mean sensitivity 83.8%, mean specificity 87.8%) and the HScore (cut-off 169, mean sensitivity 82.4%, mean specificity 87.6%). Our newly developed HLH diagnostic criteria showed inferior performance. Ferritin ≥500 µg/L had 94.0% mean sensitivity over all cohorts.
conclusionsIn this first multicenter validation study, four fulfilled HLH-2004 criteria and an HScore of 169 were suitable to diagnose secondary HLH, which will lead to rapid diagnosis and improved patient outcomes. Ferritin proved as a reliable HLH screening marker. Our results should be taken into account in clinical recommendations and in designing new studies.
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