ReviewEuropean journal of pediatrics2007
Familial and acquired hemophagocytic lymphohistiocytosis.
Review in European journal of pediatrics, 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 165 papers, 2 of them syntheses that pooled it.
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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.
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Who cites it
165 citing papers in PubMed, 2 syntheses or guidelines pooled it, 531 citations in OpenAlex.
- Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: Recommendations from the North American Consortium for Histiocytosis (NACHO).Pediatric blood & cancer · 2019Guideline
- Evidence-based diagnosis and treatment of macrophage activation syndrome in systemic juvenile idiopathic arthritis.Pediatric rheumatology online journal · 2015Pooled it
- Trial
- The IL-10/IL-6 Ratio and the Risk Score: Two Cytokines-Based Predictors for Malignancy-Associated Hemophagocytic Lymphohistiocytosis in Adults (M-HLHa).American journal of hematology · 2026Observational
- Diagnosis and treatment of familial hemophagocytic lymphohistiocytosis with isolated central nervous system involvement: A case report.Annals of hematology · 2026Article
- Severe COVID-19 Unveils Atypical Familial Hemophagocytic Lymphohistiocytosis due to a Novel HomozygousCase reports in immunology · 2026Article
- Genetic and molecular approaches for patients with familial hemophagocytic lymphohistiocytosis: a multi-center experience from Mexico.Frontiers in immunology · 2026Article
- Secondary hemophagocytic lymphohistiocytosis in pediatric patients: a single-center experience.Frontiers in pediatrics · 2026Article
- Adult-onset primary hemophagocytic syndrome with concurrent Epstein-Barr virus infection: a case report with literature review.Frontiers in oncology · 2026Article
- Central nervous system involvement in pediatric hemophagocytic lymphohistiocytosis: A single-center descriptive study of clinical features, neurodiagnostic findings, and outcomes.Clinical hematology international · 2026Article
- Perforin Expression and Natural Killer-Cell Proportion as Biomarkers in Secondary Hemophagocytic Lymphohistiocytosis.Medical science monitor : international medical journal of experimental and clinical research · 2025Observational
- Primary prostatic Burkitt's lymphoma complicated with hemophagocytic lymphohistiocytosis: a case report and literature review.Frontiers in oncology · 2025Article
- Laboratory and imaging risk factors for mortality in children with primary hemophagocytic lymphohistiocytosis.Frontiers in oncology · 2025Article
- A Rare Presentation ofCase reports in medicine · 2025Article
- Severe pneumonia caused byFrontiers in medicine · 2025Article
- Comprehensive evaluation of immune dysregulation in secondary hemophagocytic lymphohistiocytosis.Virulence · 2024Article
- Article
- Machine Learning of Laboratory Data in Predicting 30-Day Mortality for Adult Hemophagocytic Lymphohistiocytosis.Journal of clinical immunology · 2024Article
- Clinical Features of Cytokine Storm Syndrome.Advances in experimental medicine and biology · 2024Review
- Laboratory Features and Pathology of Cytokine Storm Syndromes.Advances in experimental medicine and biology · 2024Review
105 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
1 author at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition of severe hyperinflammation caused by the uncontrolled proliferation of activated lymphocytes and histiocytes secreting high amounts of inflammatory cytokines. Cardinal signs and symptoms are prolonged fever, hepatosplenomegaly and pancytopenia. Characteristic biochemical markers include elevated triglycerides, ferritin and low fibrinogen. HLH occurs on the basis of various inherited or acquired immune deficiencies. Impaired function of natural killer (NK) cells and cytotoxic T-cells (CTL) is shared by all forms of HLH. Genetic HLH occurs in familial forms (FHLH) in which HLH is the primary and only manifestation, and in association with the immune deficiencies Chédiak-Higashi syndrome 1 (CHS 1), Griscelli syndrome 2 (GS 2) and x-linked lymphoproliferative syndrome (XLP), in which HLH is a sporadic event. Most patients with acquired HLH have no known underlying immune deficiency. Both acquired and genetic forms are triggered by infections, mostly viral, or other stimuli. HLH also occurs as a complication of rheumatic diseases (macrophage activation syndrome) and of malignancies. Several genetic defects causing FHLH have recently been discovered and have elucidated the pathophysiology of HLH. The immediate aim of therapy in genetic and acquired HLH is suppression of the severe hyperinflammation, which can be achieved with immunosuppressive/immunomodulatary agents and cytostatic drugs. Patients with genetic forms have to undergo stem cell transplantation to exchange the defective immune system with normally functioning immune effector cells. In conclusion, awareness of the clinical symptoms and of the diagnostic criteria of HLH is crucial in order not to overlook HLH and to start life-saving therapy in time.
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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.