ArticleJournal of cancer research and clinical oncology2020
Hemophagocytic lymphohistiocytosis in adults: collaborative analysis of 137 cases of a nationwide German registry.
Article in Journal of cancer research and clinical oncology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 74 papers, 2 of them syntheses that pooled it.
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Who cites it
74 citing papers in PubMed, 2 syntheses or guidelines pooled 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
- Article
- Review
- Secondary Hemophagocytic Lymphohistiocytosis in a Rocky Mountain Spotted Fever Patient.Cureus · 2026Article
- Hemophagocytic Lymphohistiocytosis Secondary to Extrapulmonary Tuberculosis in an HIV Patient: A Case Report.Cureus · 2026Article
- Hemophagocytic Syndromes in Adults: Real-World Data on Mortality from a Tertiary Reference Center.Acta haematologica · 2026Article
- Long-Term Treatment With Emapalumab in an Adult Patient With Refractory Hemophagocytic Lymphohistiocytosis and Systemic Lupus Erythematosus: A Case Report.Case reports in hematology · 2026Article
- Prognostic role of prognostic nutritional index in patients with lymphoma-associated hemophagocytic lymphohistiocytosis.Future oncology (London, England) · 2025Article
- Case Report: A role for hemoadsorption in hemophagocytic lymphohistiocytosis.Frontiers in medicine · 2025Article
- Dynamic ferritin levels and survival outcomes in secondary hemophagocytic syndrome: a comprehensive analysis.Frontiers in medicine · 2025Article
- Association of albumin to urea nitrogen ratio with 30-day mortality in adult hemophagocytic lymphohistiocytosis: a retrospective cohort study.Annals of hematology · 2024Article
- Hemophagocytosis of the Hilar Pulmonary Lymph Nodes Is a More Sensitive Indicator of the Severity of COVID-19 Disease than Bone Marrow Hemophagocytosis.Diseases (Basel, Switzerland) · 2024Article
- Article
- Enhancing diagnostic precision in EBV-related HLH: a multifaceted approach usingCancer imaging : the official publication of the International Cancer Imaging Society · 2024Article
- Clinicopathological characteristics, prognostic factors, and outcomes of elderly patients with lymphoma-associated hemophagocytic lymphohistiocytosis: A multicenter analysis.Cancer medicine · 2024Article
- Clinical and Biochemical Characteristics of Hemophagocytic Lymphohistiocytosis in People Living With HIV and Disseminated Histoplasmosis at a Tertiary Hospital in Mexico.Open forum infectious diseases · 2024Article
- [Hemophagocytic lymphohistiocytosis and macrophage activation syndrome : A multidisciplinary challenge].Zeitschrift fur Rheumatologie · 2024Review
- Early hematopoietic cell transplantation for familial hemophagocytic lymphohistiocytosis in a regional treatment network in Japan.International journal of hematology · 2024Article
- Persistent Fever after COVID-19 Vaccination in a Patient with Ulcerative Colitis: A Call for Attention.GE Portuguese journal of gastroenterology · 2024Article
14 more citing papers are in PubMed but not listed here.
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Authors and funding
20 authors.
Funding
Abstract
purposeHemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome emerging from a deregulated immune response due to various triggers. In adults, systematic data are sparse, which is why recommendations on diagnosis and management have been adopted from pediatric guidelines. A nationwide clinical registry with associated consulting service as collaborative initiative of HLH-specialized pediatricians and hematologists was initiated to better characterize HLH in adults.
methodsPatients with proven or suspected HLH were registered by 44 institutions. Both HLH-2004 diagnostic criteria and the HScore (www.saintantoine.aphp.fr/score/) were used to confirm HLH diagnosis. Data referring to underlying disease, treatment, outcome, clinical presentation and laboratory findings were recorded.
resultsThe study included 137 patients and provides the first systematic data on adult HLH in Germany. Median age was 50 years with a wide range (17-87 years), 87 patients (63.5%) were male. Most common triggering diseases were infections in 61 patients (44.5%) and malignancies in 48 patients (35%). Virtually all patients had elevated ferritin concentrations, and 74% had peak concentrations greater than 10,000 µg/l. At time of analysis, 67 of 131 patients (51%) had died. Patients with malignancy-associated HLH had the shortest median survival (160 days), however no statistically significant difference between subgroups was observed (p = 0.077). Platelets under 20*10
conclusionClose multidisciplinary case consultation and cooperation is mandatory when treating adult HLH patients. Early contact with reference centers is recommended, especially in relapsing or refractory disease.
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