ArticleOrphanet journal of rare diseases2015
Clinical characteristics and prognostic factors of adult hemophagocytic syndrome patients: a retrospective study of increasing awareness of a disease from a single-center in China.
Article in Orphanet journal of rare diseases, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 62 papers, 3 of them syntheses that pooled it.
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Who cites it
62 citing papers in PubMed, 3 syntheses or guidelines pooled it, 120 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
- Trial
- Elevated serum myoglobin levels at hospital admission and the risk of early death among patients with hemophagocytic lymphohistiocytosis: evidence from 155 pediatric patients.Annals of hematology · 2020Trial
- Review
- IFN-γ Driven Hepatic Injury Exacerbates Mortality in NK/T-Cell Lymphoma-Associated Hemophagocytic Lymphohistiocytosis.Cancer medicine · 2026Article
- Hemophagocytic Syndromes in Adults: Real-World Data on Mortality from a Tertiary Reference Center.Acta haematologica · 2026Article
- Nomogram for predicting the 30- and 90-day prognoses of hemophagocytic lymphohistiocytosis patients: a multicenter and retrospective study.Annals of hematology · 2025Article
- Article
- Clinical characteristics and prognostic factors in patients with fever of unknown origin who developed secondary haemophagocytic lymphohistiocytosis.Annals of hematology · 2025Article
- Pathogenesis, Diagnosis, and Management of Cytokine Release Syndrome in Patients with Cancer: Focus on Infectious Disease Considerations.Current oncology (Toronto, Ont.) · 2025Review
- A Case Report of Hemophagocytic Syndrome.Cureus · 2025Article
- Review
- A convenient and practical index for predicting the induction response in adult patients with hemophagocytic lymphohistiocytosis: ferritin/platelet ratio.Annals of hematology · 2024Review
- Liver Dysfunction in Adult Hemophagocytic Lymphohistiocytosis: A Narrative Review.Advances in therapy · 2024Review
- Patients with Hemophagocytic Lymphohistiocytosis Who Need Intensive Care Can Be Successfully Rescued by Timely Using Etoposide-Based HLH Regimens.International journal of general medicine · 2024Article
- Hemophagocytic lymphohistiocytosis with jaundice as first manifestation: A case report.World journal of clinical cases · 2023Article
- A Case Report of Aggressive Post-Infectious Hemophagocytic Lymphohistiocytosis in an Immunocompetent Adult.Cureus · 2023Article
- A novel prognosis-prediction model based on coagulation indicators in secondary hemophagocytic lymphohistiocytosis.Annals of hematology · 2023Article
2 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
11 authors at 6 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundHemophagocytic lymphohistiocytosis (HLH) is a relatively rare but life-threatening disease with confusing clinical manifestations, rapidly deteriorating health, high morbidity and mortality.
methodsTo improve the recognition as well as understanding of this disorder, we analyzed clinical characteristics and prognostic factors from 85 adult patients diagnosed with HLH in our hospital from April 2005 to June 2014.
resultsPatients with HLH displayed variable clinical markers across a wide spectrum. These included fever and hyperferritinemia (100%), elevated lactate dehydrogenase (LDH) (98.8%), two or three cytopenia (92.2%), splenomegaly (72.9%), hypofibrinogenemia (69.4%), hypertriglyceridemia (64.7%), hemophagocytosis (51.7%), and hepatomegaly (24.7%). Patients with active Epstien-Barr Virus (EBV) infection had a median overall survival (OS) of 65 days. Those displaying malignancy had very poor survival (median OS: 40 days). However, patients in rheumatic and non-EBV infection groups had relatively superior prognosis (not reached). Univariate analysis showed that Fibrinogen (Fbg) <1.5 g/L, platelet number (PLT) <40 × 10(9)/L and LDH ≥1000 U/L were factors that negatively affected survival (P = 0.004, 0.000, 0.002). Multivariate analysis showed that PLT <40 × 10(9)/L was the independent adverse factor (HR = 0.350, 95% CI: 0.145-0.844, P = 0.019).
conclusionsHLH had very complex clinical manifestations and high death rate. Patients with active EBV infection, malignancy, Fbg <1.5 g/L, PLT <40 × 10(9)/L and LDH ≥1000 U/L had high risk of death as well as inferior survival, and these patients require systemic targeted treatments as early as possible.
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