ArticleHemaSphere2022
Temporal Trends in the Incidence of Hemophagocytic Lymphohistiocytosis: A Nationwide Cohort Study From England 2003-2018.
Article in HemaSphere, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
26 citing papers in PubMed, 32 citations in OpenAlex.
- Clinical-Pathological Conference Series from the Medical University of Graz : Case No 186: A 33-year-old plumber with fever and acute liver injury.Wiener klinische Wochenschrift · 2026Article
- Secondary hemophagocytic lymphohistiocytosis following bacterial pneumonia managed with dexamethasone monotherapy: a case report.Annals of medicine and surgery (2012) · 2026Article
- The HLH-Risk-Calculator is a machine learning-based tool to predict course & mortality of secondary hemophagocytic lymphohistiocytosis.Intensive care medicine · 2026Article
- Hemophagocytic Lymphohistiocytosis Triggered byClinical case reports · 2026Article
- Cytokine storm.Nature reviews. Disease primers · 2026Review
- Secondary Hemophagocytic Lymphohistiocytosis as the Initial Presentation of Metastatic Carcinoma of Likely Colorectal Origin.Journal of Brown hospital medicine · 2026Article
- Hemophagocytic lymphohistiocytosis: do we have a solution for TMI (too much inflammation)?Hematology. American Society of Hematology. Education Program · 2025Review
- Nomogram for predicting the 30- and 90-day prognoses of hemophagocytic lymphohistiocytosis patients: a multicenter and retrospective study.Annals of hematology · 2025Article
- Article
- Article
- Clinical features, course, and risk factors of infection-associated secondary hemophagocytic lymphohistiocytosis.Infection · 2025Article
- Article
- Fungal-Induced Hemophagocytic Lymphohistiocytosis: A Literature Review in Non-HIV Populations.Journal of fungi (Basel, Switzerland) · 2025Review
- HHV-8-Associated Hemophagocytic Lymphohistiocytosis in a HIV-Negative and Nontransplant Man: A Case Report and Literature Review.Case reports in infectious diseases · 2025Article
- Pancytopenia With Subsequent Diagnosis of Hemophagocytic Lymphohistiocytosis in a Middle-Aged Male.Case reports in oncological medicine · 2025Article
- Risk Factors of Hemophagocytic Lymphohistiocytosis in Adults with Fever of Unknown Origin: A Retrospective Study.International journal of general medicine · 2025Article
- Epidemiological, clinical characteristics and prognostic factors analysis of adult patients with hemophagocytic lymphohistiocytosis in a Chinese hospital.Frontiers in immunology · 2025Article
- Macrophage activation syndrome successfully treated with eculizumab and emapalumab: a case report.Frontiers in immunology · 2025Article
- Prequalification of genome-based newborn screening for severe childhood genetic diseases through federated training based on purifying hyperselection.American journal of human genetics · 2024Article
- Liver Dysfunction in Adult Hemophagocytic Lymphohistiocytosis: A Narrative Review.Advances in therapy · 2024Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
11 authors at 4 institutions in 1 country.
Funding
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is rare, results in high mortality, and is increasingly being diagnosed. We aimed to quantify the incidence of diagnosed HLH and examine temporal trends in relation to age and associated diseases. Using national linked electronic health data from hospital admissions and death certification cases of HLH that were diagnosed in England between January 1, 2003, and December 31, 2018. We calculated incidence rates of diagnosed HLH per million population by calendar year, age group, sex, and associated comorbidity (hematological malignancy, inflammatory rheumatological or bowel diseases [IBD]). We modeled trends in incidence and the interactions between calendar year, age, and associated comorbidity using Poisson regression. There were 1674 people with HLH diagnosed in England between 2003 and 2018. The incidence rate quadrupled (incidence rate ratio [IRR] 2018 compared to 2003: 3.88, 95% confidence interval [CI] 2.91 to 5.28), increasing 11% annually (adjusted IRR 1.11, 95% CI 1.09 to 1.12). There was a transition across age groups with greater increases in those aged 5-14 years of HLH associated with rheumatological disease/IBD compared with hematological malignancy, with similar increases in HLH associated with both comorbidities for those 15-54, and greater increases in HLH associated with hematological malignancies for those 55 years and older. The incidence of HLH in England has quadrupled between 2003 and 2018. Substantial variation in the incidence occurred with inflammatory rheumatological diseases/IBD-associated HLH increasing more among the younger age groups, whereas in older age groups, the largest increase was seen with hematological malignancy-associated HLH.
Identifiers
What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.