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Abstracts tagged "macrophage activation syndrome and natural killer (NK) cells"

  • Abstract Number: 2772 • ACR/ARHP Annual Meeting 2017

    Unbound IL-18 Distinguishes Human Macrophage Activation Syndrome from Familial Hemophagocytic Lymphohistiocytosis and Affects Innate Versus Adaptive Murine Lymphocytes Differently

    Paul Tsoukas1, Eric Weiss2, Dirk Holzinger3, Charlotte Girard4, Dirk Foell5, Alexei A. Grom6, Sandra Ammann7, Stephan Ehl7, Eduardo Schiffrin8, Adriana Almeida de Jesus9, Raphaela Goldbach-Mansky9, Cem Gabay10 and Scott Canna11, 1Pediatric Rheumatology, Children’s Hospital of Pittsburgh, Pittsburgh, PA, 2RK Mellon Institute, Children’s Hospital of Pittsburgh, Pittsburgh, PA, 3Department of Pediatric Rheumatology and Immunology, University of Muenster, Muenster, Germany, 4Division of Rheumatology, Department of Internal Medicine Specialties, University Hospital of Geneva, Geneva, Switzerland, 5University of Muenster, Muenster, Germany, 6Pediatric Rheumatology, Cincinnati Children’s Hospital, Cincinnati, OH, 7Center for Chronic Immunodeficiency, Faculty of Medicine, University of Freiburg, Freiburg, Germany, 8AB2 Bio, Lausanne, Switzerland, 9Translational Autoinflammatory Disease Studies (TADS), Laboratory of Clinical Investigation and Microbiology (LCIM), NIAID/NIH, Bethesda, MD, 10SCQM, Geneva, Switzerland, Geneva, Switzerland, 11Richard King Mellon Foundation Institute for Pediatric Research, Children's Hospital of Pittsburgh, Pittsburrgh, PA

    Background/Purpose: Persistently and extremely elevated serum IL-18 has been associated with Macrophage Activation Syndrome (MAS). Chronic IL-18 is hypothesized to contribute to excessive interferon (IFN)-g…
  • Abstract Number: 311 • ACR/ARHP Annual Meeting 2014

    Inhibition of Natural Killer (NK) Cell Cytotoxicity By Interleukin-6 (IL-6): Implications for the Pathogenesis of Macrophage Activation Syndrome

    Loredana Cifaldi1, Giusi Prencipe2, Ivan Caiello2, Claudia Bracaglia2, Raffaele Strippoli3 and Fabrizio De Benedetti Sr.2, 1Paediatric Haematology/Oncology, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy, 2Department of Pediatric Medicine, Division of Rheumatology, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy, 3Cellular Biotechnologies and Haematology, Sapienza Rome University, Rome, Italy

    Background/Purpose: MAS occurs frequently in patients with active systemic juvenile idiopathic arthritis(s-JIA) and because of the similarities with Haemophagocytic Lymphohistiocytosis (HLH) is classified among secondary…
  • Abstract Number: 2655 • ACR/ARHP Annual Meeting 2012

    Rapid and Effective Response to Immunosuppression in Treating Macrophage Activation Syndrome Associated with a Heterozygous Dominant Negative Mutation in RAB27a Leading to Decreased Cytolytic Activity

    Randy Q. Cron1, Mingce Zhang2, Christina J. Bemrich-Stolz2 and Timothy Beukelman1, 1Pediatric Rheumatology, University of Alabama at Birmingham, Birmingham, AL, 2Pediatrics, Univ of Alabama-Birmingham, Birmingham, AL

    Background/Purpose: Hemophagocytic lymphohistiocytosis (HLH) is an often fatal disorder of infancy resulting from homozygous mutations in proteins involved in cytolysis (e.g. MUNC13-4, RAB27a, Perforin 1,…

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