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

Abstract Number: 2772 • 2017 ACR/ARHP Annual Meeting
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 • 2014 ACR/ARHP Annual Meeting
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 • 2012 ACR/ARHP Annual Meeting
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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