ArticleThe Journal of allergy and clinical immunology2019
IFN-γ and CD25 drive distinct pathologic features during hemophagocytic lymphohistiocytosis.
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Article in The Journal of allergy and clinical immunology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 42 papers.
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42citing papers in PubMed
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Who cites it
42 citing papers in PubMed, 60 citations in OpenAlex.
- Emapalumab in primary haemophagocytic lymphohistiocytosis and the pathogenic role of interferon gamma: A pharmacometric model-based approach.British journal of clinical pharmacology · 2022Trial
- Emapalumab plus conventional therapy with or without ruxolitinib for pediatric hemophagocytic lymphohistiocytosis: a single center retrospective study.Immunologic research · 2026Article
- Chimeric antigen receptor T-cell therapies related to immune effector cell-associated hemophagocytic lymphohistiocytosis-like syndrome: Diagnosis, high-risk factors, and management.Chinese medical journal · 2026Review
- T-ing up the storm: pathogenic cycling lymphocytes in the biology of macrophage activation syndrome.Pediatric rheumatology online journal · 2025Review
- The Microbiome Modifies Manifestations of Hemophagocytic Lymphohistiocytosis in Perforin-Deficient Mice.European journal of immunology · 2025Article
- Directed disruption of IL2 aggregation and receptor binding sites produces designer biologics with enhanced specificity and improved production capacity.Computational and structural biotechnology journal · 2025Article
- Real-world treatment patterns and outcomes in patients with primary hemophagocytic lymphohistiocytosis treated with emapalumab.Blood advances · 2024Article
- Early hematopoietic cell transplantation for familial hemophagocytic lymphohistiocytosis in a regional treatment network in Japan.International journal of hematology · 2024Article
- Case report: emapalumab treatment for a pediatric Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) patient with cytokine storm enabling allogeneic hematopoietic cell transplantation.Translational pediatrics · 2024Article
- Combined emapalumab and ruxolitinib in patients with haemophagocytic Lymphohistiocytosis.Blood cancer journal · 2024Article
- Recombinant IFN-γ1b Treatment in a Patient with Inherited IFN-γ Deficiency.Journal of clinical immunology · 2024Article
- Rapid Diagnosis of Hemophagocytic Lymphohistiocytosis Triggered by Disseminated BCG Infection in Infants With Severe Combined Immunodeficiency: Case Report.Open forum infectious diseases · 2023Article
- Mendelian Susceptibility to Mycobacterial Disease (MSMD): Clinical, Immunological, and Genetic Features of 22 Patients from 15 Moroccan Kindreds.Journal of clinical immunology · 2023Article
- When to suspect inborn errors of immunity in Epstein-Barr virus-related lymphoproliferative disorders.Clinical microbiology and infection : the official publication of the European Society of Clinical Microbiology and Infectious Diseases · 2023Review
- Severe Mycobacterial Immune Reconstitution Inflammatory Syndrome (IRIS) in Advanced Human Immunodeficiency Virus (HIV) Has Features of Hemophagocytic Lymphohistiocytosis and Requires Prolonged Immune Suppression.Clinical infectious diseases : an official publication of the Infectious Diseases Society of America · 2023Article
- Insights into the cellular pathophysiology of familial hemophagocytic lymphohistiocytosis.Frontiers in immunology · 2023Review
- The hyperinflammatory spectrum: from defects in cytotoxicity to cytokine control.Frontiers in immunology · 2023Review
- HBM4EU Diisocyanates Study-Research Protocol for a Collaborative European Human Biological Monitoring Study on Occupational Exposure.International journal of environmental research and public health · 2022Article
- Context-dependent effects of IL-2 rewire immunity into distinct cellular circuits.The Journal of experimental medicine · 2022Article
- TIM3+ TRBV11-2 T cells and IFNγ signature in patrolling monocytes and CD16+ NK cells delineate MIS-C.The Journal of experimental medicine · 2022Article
Corrections and comments
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Authors and funding
10 authors at 5 institutions in 4 countries.
Stéphanie Humblet-BaronVIB Center for Brain & Disease Research, Leuven, Belgium; KU Leuven-University of Leuven, Department of Microbiology and Immunology, Leuven, Belgium.
Dean FranckaertVIB Center for Brain & Disease Research, Leuven, Belgium; KU Leuven-University of Leuven, Department of Microbiology and Immunology, Leuven, Belgium.
James DooleyVIB Center for Brain & Disease Research, Leuven, Belgium; KU Leuven-University of Leuven, Department of Microbiology and Immunology, Leuven, Belgium.
Fatima AilalClinical Immunology Unit, Casablanca Children's Hospital, Ibn Rochd Laboratoire LICIA d'Immunologie Clinique, Inflammation et Allergie, Medical School, Hassan II University, Casablanca, Morocco.
Aziz BousfihaClinical Immunology Unit, Casablanca Children's Hospital, Ibn Rochd Laboratoire LICIA d'Immunologie Clinique, Inflammation et Allergie, Medical School, Hassan II University, Casablanca, Morocco.
Caroline DeswarteLaboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Imagine Institute, Necker Hospital for Sick Children, Paris, France; Paris Descartes University, Paris, France.
Carmen Oleaga-QuintasLaboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Imagine Institute, Necker Hospital for Sick Children, Paris, France; Paris Descartes University, Paris, France.
Jean-Laurent CasanovaLaboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Imagine Institute, Necker Hospital for Sick Children, Paris, France; Paris Descartes University, Paris, France; St Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, Rockefeller University, New York, NY; Howard Hughes Medical Institute, New York, NY; Pediatric Hematology-Immunology Unit, Assistance Publique-Hôpitaux de Paris AP-HP, Necker Hospital for Sick Children, Paris, France.
Jacinta BustamanteLaboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Imagine Institute, Necker Hospital for Sick Children, Paris, France; Paris Descartes University, Paris, France; St Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, Rockefeller University, New York, NY; Center for the Study of Primary Immunodeficiencies, Assistance Publique-Hôpitaux de Paris AP-HP, Necker Hospital for Sick Children, Paris, France.
Adrian ListonVIB Center for Brain & Disease Research, Leuven, Belgium; KU Leuven-University of Leuven, Department of Microbiology and Immunology, Leuven, Belgium. Electronic address: adrian.liston@gmail.com.
Institut des Maladies Génétiques Imagine · FRKU Leuven · BEUniversity of Hassan II Casablanca · MAHoward Hughes Medical Institute · USVIB-KU Leuven Center for Brain & Disease Research · BE
Funding
Developing, Demonstrating, and Disseminating Innovative Programs to Achieve Translational SuccessUL1TR001866 · NCATS · ROCKEFELLER UNIVERSITY · PI COLLER, BARRY, KRUEGER, JAMES G · 2016 to 2025
$40.6MTransforming Translational Science and Education to Benefit Human HealthUL1TR000043 · NCATS · ROCKEFELLER UNIVERSITY · PI COLLER, BARRY · 2012 to 2015
$26.8MGenome-Wide Dissection of Mendelian Susceptibility to Mycobacterial DiseaseR37AI095983 · NIAID · ROCKEFELLER UNIVERSITY · PI CASANOVA, JEAN-LAURENT · 2011 to 2020
$4.6MA genetic dissection of Mendelian susceptibility to mycobacterial diseases (MSMD)R01AI089970 · NIAID · ROCKEFELLER UNIVERSITY · PI CASANOVA, JEAN-LAURENT · 2010 to 2014
$2.5MNCATS NIH HHS UL1 TR000043NCATS NIH HHS UL1 TR001866NIAID NIH HHS R01 AI089970NIAID NIH HHS R37 AI095983
Abstract
backgroundInflammatory activation of CD8
objectiveThe mechanism linking CD8
methodsIn addition to reporting a complete autosomal recessive IFN-γ receptor 1-deficient patient with multiple aspects of HLH pathology, we used the mouse model of perforin (Prf1)
resultsWe found a striking dichotomy between the mechanistic basis of the hematologic and inflammatory components of CD8
conclusionThese results synthesize the competing mechanistic models of HLH pathology into a dichotomous pathogenesis driven through discrete pathways. A holistic model provides a new paradigm for understanding HLH and, more broadly, the consequences of CD8
Indexed as
AnimalsCD8-Positive T-LymphocytesConsanguinityDisease Models, AnimalFemaleHumansInfantInflammationInterferon-gammaInterferon gamma ReceptorInterleukin-2 Receptor alpha SubunitLymphocyte ActivationLymphocytic ChoriomeningitisLymphocytic choriomeningitis virusLymphohistiocytosis, HemophagocyticMiceInterferon-gammaInterferon gamma ReceptorInterleukin-2 Receptor alpha SubunitPerforinPRF1 protein, humanReceptors, InterferonCD25CD8(+) T-cell hyperactivationhemophagocytic lymphohistiocytosisIFN-γ
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