ReviewIndian journal of pediatrics2016
Current Updates on Classification, Diagnosis and Treatment of Hemophagocytic Lymphohistiocytosis (HLH).
Review in Indian journal of pediatrics, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 33 papers.
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Who cites it
33 citing papers in PubMed, 78 citations in OpenAlex.
- Early management of hemophagocytic lymphohistiocytosis/macrophage activation syndrome: a systematic literature review.EULAR rheumatology open · 2026Review
- Article
- Construction of a forest plot prediction model based on Lasso regression for Epstein-Barr virus associated hemophagocytic lymphohistiocytosis in children.Italian journal of pediatrics · 2026Article
- Malignancy-Associated Hemophagocytic Lymphohistiocytosis: An Experience of 15 Years in Polish Pediatric Hematology Centers.Journal of blood medicine · 2026Article
- Acquired Hypolipoproteinemia and Hemophagocytic Lymphohistiocytosis: A Case Series and Review.Hematology reports · 2025Review
- Assessment of Hemophagocytic Lympho-Histiocytosis (HLH) in the Setting of Adult Acute Liver Failure.Journal of viral hepatitis · 2025Article
- A case report of hemophagocytic syndrome induced byFrontiers in immunology · 2025Article
- A hidden enemy: Understanding the hemophagocytic syndrome in children under five years of age in a high-complexity institution in southwestern ColombiaBiomedica : revista del Instituto Nacional de Salud · 2024Article
- Comprehensive evaluation of immune dysregulation in secondary hemophagocytic lymphohistiocytosis.Virulence · 2024Article
- Case Report: A Case of Complicated Typhoid Fever with Rhabdomyolysis and Hemophagocytic Lymphohistiocytosis.The American journal of tropical medicine and hygiene · 2024Article
- A novel prognosis-prediction model based on coagulation indicators in secondary hemophagocytic lymphohistiocytosis.Annals of hematology · 2023Article
- Wolman disease presenting with hemophagocytic lymphohistiocytosis syndrome and a novel LIPA gene variant: a case report and review of the literature.Journal of medical case reports · 2023Review
- Disseminated tuberculosis in rare association with hemophagocytic lymphocytosis - A case report from central India.Heliyon · 2023Article
- Serum ferritin is a good indicator for predicting the efficacy of adult HLH induction therapy.Annals of medicine · 2022Article
- B-cell lymphoma-associated hemophagocytic lymphohistiocytosis: A case report.Oncology letters · 2022Article
- Inactivated COVID-19 vaccine triggering hemophagocytic lymphohistiocytosis in an immunocompetent adult - A case report.Journal of clinical and translational research · 2022Article
- [Characteristic and prognosis of patients with non-EBV infection-associated hemophagocytic lymphohistiocytosis].Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2022Article
- CD8 + T Cells Exhibit an Exhausted Phenotype in Hemophagocytic Lymphohistiocytosis.Journal of clinical immunology · 2021Article
- Molecular Genetics Diversity of Primary Hemophagocytic Lymphohistiocytosis among Polish Pediatric Patients.Archivum immunologiae et therapiae experimentalis · 2021Article
- Pediatric hemophagocytic lymphohistiocytosis: A rarely diagnosed entity in a developing country.BMC pediatrics · 2021Article
Corrections and comments
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Authors and funding
3 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a life threatening hyperinflammatory syndrome characterized by excessive activation of macrophages and T cells resulting from defective cytotoxicity. Severe hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and histiocytes (macrophages) secreting high amounts of inflammatory cytokines threatens the life of the patient and may lead to death unless arrested by appropriate treatment. HLH can be caused either by certain underlying genetic diseases (familial HLH), or may also occur due to particular triggers in patients with no known inherited disorder (acquired HLH). Due to life threatening nature of the disease, early diagnosis and initiation of immunosuppressive therapy is extremely important. HLH diagnosis is based on constellation of clinical manifestations and laboratory parameters which often overlap with those of severe infection or sepsis. Identification of patients with familial HLH and their underlying genetic defects requires specialized laboratory tests and is important for predicting relapses and planning early therapeutic hematopoietic stem cell transplantation (HSCT). A high suspicion and thorough clinical, immunological and genetic work-up is required for diagnosis of HLH. Prompt initiation of adequate treatment is essential for the survival. Substantial progress has been made in exploring the complex cause and pathophysiology of HLH and also in management of HLH patients.
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