ReviewArthritis research & therapy2012
Recent advances in the diagnosis and treatment of hemophagocytic lymphohistiocytosis.
Review in Arthritis research & therapy, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 70 papers, 1 of them a synthesis that pooled it.
What it found
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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
70 citing papers in PubMed, 1 synthesis or guideline pooled it, 159 citations in OpenAlex.
- Differential Diagnosis of Hemophagocytic Syndrome byJournal of healthcare engineering · 2022Pooled it
- [Visceral leishmaniasis with suspected autoimmune disease: A report of 2 cases].Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences · 2026Article
- Genotoxicity profiling reveals distinct platform-and cell type-specific effects in therapeutic gene editing for genetic hyperinflammation.Cell stem cell · 2026Article
- A Narrative Review of Drug-Induced Haemophagocytic Lymphohistiocytosis: A Rare but Relevant Differential Diagnosis.International archives of allergy and immunology · 2026Review
- Promising Targeted Therapies for Hemophagocytic Lymphohistiocytosis: A Translational Perspective Based on Immunopathology.Clinical reviews in allergy & immunology · 2025Review
- Deep insight into cytokine storm: from pathogenesis to treatment.Signal transduction and targeted therapy · 2025Review
- Predicting 30-day mortality in hemophagocytic lymphohistiocytosis: clinical features, biochemical parameters, and machine learning insights.Annals of hematology · 2025Article
- Severe pneumonia caused byFrontiers in medicine · 2025Article
- Hemophagocytic lymphohistiocytosis: current treatment advances, emerging targeted therapy and underlying mechanisms.Journal of hematology & oncology · 2024Review
- Diarrhea as the Initial Presentation in a Patient With HIV Diagnosed With Hodgkin's Lymphoma.Cureus · 2024Article
- Impaired STING Activation Due to a Variant in the E3 Ubiquitin Ligase AMFR in a Patient with Severe VZV Infection and Hemophagocytic Lymphohistiocytosis.Journal of clinical immunology · 2024Article
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- Prognostic death factors in secondary hemophagocytic lymphohistiocytosis children with multiple organ dysfunction syndrome receiving continuous renal replacement therapy: A multicenter prospective nested case-control study.Therapeutic apheresis and dialysis : official peer-reviewed journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy · 2022Article
- Hemophagocytic LymphohistiocytosisBalkan medical journal · 2022Article
- Treatment of an HLH-mimic disease based on HAVCR2 variants with absent TIM-3 expression.Blood advances · 2022Article
- Inactivated COVID-19 vaccine triggering hemophagocytic lymphohistiocytosis in an immunocompetent adult - A case report.Journal of clinical and translational research · 2022Article
- Review
10 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
7 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening disease of severe hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and macrophages secreting high amounts of inflammatory cytokines. It is a frequent manifestation in patients with predisposing genetic defects, but can occur secondary to various infectious, malignant, and autoimmune triggers in patients without a known genetic predisposition. Clinical hallmarks are prolonged fever, cytopenias, hepatosplenomegaly, and neurological symptoms, but atypical variants presenting with signs of chronic immunodeficiency are increasingly recognized. Impaired secretion of perforin is a key feature in several genetic forms of the disease, but not required for disease pathogenesis. Despite progress in diagnostics and therapy, mortality of patients with severe HLH is still above 40%. Reference treatment is an etoposide-based protocol, but new approaches are currently explored. Key for a favorable prognosis is the rapid identification of an underlying genetic cause, which has been facilitated by recent immunological and genetic advances. In patients with predisposing genetic disease, hematopoietic stem cell transplantation is performed increasingly with reduced intensity conditioning regimes. Current research aims at a better understanding of disease pathogenesis and evaluation of more targeted approaches to therapy, including anti-cytokine antibodies and gene therapy.
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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.