SynthesisExperimental & molecular medicine2024
Immunopathology of and potential therapeutics for secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome: a translational perspective.
Synthesis in Experimental & molecular medicine, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 31 papers.
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Who cites it
31 citing papers in PubMed, 31 citations in OpenAlex.
- Review
- Development of a machine learning-based assistive diagnostic model for Epstein‑Barr virus‑associated hemophagocytic lymphohistiocytosis in pediatric patients.Translational pediatrics · 2026Article
- Bone marrow hemophagocytosis in patients with hematological malignancies and COVID-19 infections: mimicry or secondary HLH?Journal of hematopathology · 2026Article
- Development and internal validation of a nomogram for predicting 30-day mortality in older patients with hemophagocytic lymphohistiocytosis.BMC geriatrics · 2026Article
- Paradoxical Hemophagocytic Lymphohistiocytosis to Stevens-Johnson Syndrome - Toxic Epidermal Necrolysis- A rare immune phenomenon.Journal of postgraduate medicine · 2026Article
- Development and validation of an early risk-stratification model for hemophagocytic lymphohistiocytosis in severe fever with thrombocytopenia syndrome.PLoS neglected tropical diseases · 2026Article
- Secondary Hemophagocytic Lymphohistiocytosis in a Rocky Mountain Spotted Fever Patient.Cureus · 2026Article
- Lysine-11 ubiquitination drives type-I/III interferon induction by cGAS-STING and Toll-like receptors 3 and 4.Nature cell biology · 2026Article
- Case Report: Hemophagocytic lymphohistiocytosis in an infant associated with cytomegalovirus infection and X-linked primary immunodeficiency.Frontiers in immunology · 2026Article
- Adult-Onset Still's Disease in a Patient with Macrophage Activation Syndrome and Pre-Disseminated Intravascular Coagulation: A Case Report and Literature Review.Journal of inflammation research · 2026Article
- [Clinical analysis of nine cases of hemophagocytic lymphohistiocytosis during pregnancy: characteristics, therapeutic strategies, and maternal-infant outcomes].Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2025Article
- Endothelial dysfunction and proinflammatory state determine severe hematotoxicity and inferior outcome of CAR-T therapy.HemaSphere · 2025Article
- Article
- Hyperferritinemia and Macrophage Activation Syndrome in Septic Shock: Recent Advances with a Pediatric Focus (2020-2025).Children (Basel, Switzerland) · 2025Review
- Article
- Rare early hematogenous disseminated tuberculosis inducing hemophagocytic syndrome in conflict treatment.BMC infectious diseases · 2025Article
- NLRP3 inflammasome-driven hemophagocytic lymphohistiocytosis occurs independent of IL-1β and IL-18 and is targetable by BET inhibitors.Science advances · 2025Article
- Promising Targeted Therapies for Hemophagocytic Lymphohistiocytosis: A Translational Perspective Based on Immunopathology.Clinical reviews in allergy & immunology · 2025Review
- Systematic identification of cancer-type-specific drugs based on essential genes and validations in lung adenocarcinoma.Briefings in bioinformatics · 2025Article
- Accelerating and protective effects toward cancer growth in cGAS and FcgRIIb deficient mice, respectively, an impact of macrophage polarization.Inflammation research : official journal of the European Histamine Research Society ... [et al.] · 2025Article
Corrections and comments
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome (sHLH/MAS) is a life-threatening immune disorder triggered by rheumatic disease, infections, malignancies, or medications. Characterized by the presence of hemophagocytic macrophages and a fulminant cytokine storm, sHLH/MAS leads to hyperferritinemia and multiorgan failure and rapidly progresses to death. The high mortality rate and the lack of specific treatments necessitate the development of a new drug. However, the complex and largely unknown immunopathologic mechanisms of sHLH/MAS, which involve dysfunction of various immune cells, diverse etiologies, and different clinical contexts make this effort challenging. This review introduces the terminology, diagnosis, and clinical features of sHLH/MAS. From a translational perspective, this review focuses on the immunopathological mechanisms linked to various etiologies, emphasizing potential drug targets, including key molecules and signaling pathways. We also discuss immunomodulatory biologics, existing drugs under clinical evaluation, and novel therapies in clinical trials. This systematic review aims to provide insights and highlight opportunities for the development of novel sHLH/MAS therapeutics.
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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.