ArticleRheumatology (Oxford, England)2019
Haemophagocytic lymphohisticytosis-an underrecognized hyperinflammatory syndrome.
Article in Rheumatology (Oxford, England), 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers.
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Who cites it
27 citing papers in PubMed, 43 citations in OpenAlex.
- Complexities in the Diagnostic Evaluation and Treatment of Infant Hemophagocytic Lymphohistiocytosis.Hematology reports · 2026Article
- Trends in Mortality Rates From Hemophagocytic Lymphohistiocytosis in the United States From 2010 to 2023.Cureus · 2026Article
- An Unusual Presentation of Secondary Hemophagocytic Lymphohistiocytosis With Melena: A Case Report.Cureus · 2026Article
- Article
- Hemophagocytic Lymphohistiocytosis in HIV/AIDS: Who Fired the First Shot, the Virus or Its Companions?Cureus · 2025Article
- Body mass index and hemophagocytic lymphohistiocytosis: a risk assessment in HIV-positive leukemia patients.Annals of medicine and surgery (2012) · 2025Review
- Haemophagocytic lymphohistiocytosis in critical care.BJA education · 2025Review
- Brucella-associated hemophagocytic syndrome: case report of a potentially life-threatening condition and literature review.Frontiers in immunology · 2025Review
- Turmeric andJournal of inflammation research · 2025Article
- Haemophagocytic Lymphohistiocytosis as an Initial Presentation of Undiagnosed Systemic Lupus Erythematosus.European journal of case reports in internal medicine · 2025Article
- [Hemophagocytic lymphohistiocytosis and macrophage activation syndrome : A multidisciplinary challenge].Zeitschrift fur Rheumatologie · 2024Review
- Hemophagocytic Lymphohistiocytosis: A Case Report.Cureus · 2024Article
- Cytokine Storm and Sepsis-Induced Multiple Organ Dysfunction Syndrome.Advances in experimental medicine and biology · 2024Review
- Improved hemophagocytic lymphohistiocytosis index predicts prognosis of adult Epstein-Barr virus-associated HLH patients.Annals of medicine · 2023Article
- Article
- Mortality and Associated Causes in Hemophagocytic Lymphohistiocytosis: A Multiple-Cause-of-Death Analysis in France.Journal of clinical medicine · 2023Article
- Excessive IL-10 and IL-18 trigger hemophagocytic lymphohistiocytosis-like hyperinflammation and enhanced myelopoiesis.The Journal of allergy and clinical immunology · 2022Article
- Histiocytic Glomerulopathy Associated With Hemophagocytic Lymphohistiocytosis.Kidney medicine · 2022Article
- Haemophagocytic lymphohistiocytosis in pregnancy.Clinical medicine (London, England) · 2021Article
- Defining the scourge of COVID-19 hyperinflammatory syndrome.The Lancet. Rheumatology · 2020Article
Corrections and comments
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Authors and funding
3 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Haemophagocytic lymphohisticytosis (HLH) is a syndrome of uncontrolled, severe systemic inflammation (hyperinflammation) arising either from a genetic immune system defect [primary (pHLH)] or triggered as a complication of malignancy, infection, or rheumatologic disease [secondary (sHLH)]. Patients with HLH often have non-specific symptoms and become progressively and critically unwell, with fever, cytopenia and multi-organ failure. Untreated, HLH is almost universally fatal, but even when treated, mortality is high, particularly when HLH complicates malignancy. HLH is managed with immunosuppression, and this can seem difficult to justify in such unwell patients. This review aims to examine the diagnostic and treatment challenges posed by sHLH and to improve recognition among rheumatologists who, being expert in the management of multisystem diseases and in the use of immunosuppression, are ideally placed to deliver care and build an evidence base for better disease characterization and treatment.
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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.