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POS0339 POINTS TO CONSIDER AT THE EARLIEST STAGES OF THE DIAGNOSIS AND MANAGEMENT OF HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS/MACROPHAGE ACTIVATION SYNDROME (HLH/MAS)
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  1. B. Shakoory1,
  2. A. Geerlinks2,
  3. M. Wilejto3,
  4. K. Kernan4,
  5. E. Demirkaya3,
  6. A. Ravelli5,
  7. R. Sinha6,
  8. R. Goldbach-Mansky1,
  9. F. De Benedetti7,
  10. R. Marsh8,
  11. S. Canna9
  12. on behalf of EULAR/ACR HLH/MAS Working Group
  1. 1NIAID/NIH, Translational Autoinflammatory Disease Section, Bethesda, United States of America
  2. 2London Health Sciences Centre, Hematology/Oncology, London, Canada
  3. 3London Health Sciences Centre, Rheumatology, London, Canada
  4. 4University of Pittsburgh, Critical Care Medicine, Pittsburgh, United States of America
  5. 5Instiuto Gaslini, Rheumatology, Genoa, Italy
  6. 6Systemic JIA Foundation, Head, Cincinnati, United States of America
  7. 7Ospedale Bambino Gesu, Rheumatology, Rome, Italy
  8. 8Cincinnati Children’s Hospital Medical Center, Bone Marrow Transplantation and Immune Deficiency, Cincinnati, United States of America
  9. 9The Children’s Hospital of Philadelphia, Rheumatology & Immune Dysregulation, Philadelphia, United States of America

Abstract

Background Hemophagocytic Lymphohistiocytosis (HLH) and Macrophage Activation Syndrome (MAS) are life-threatening systemic hyperinflammatory syndromes that occur in many contexts and are often called by many names. They nevertheless can progress rapidly, and early identification and management are critical for preventing organ failure and mortality.

Objectives The purpose of this effort was to develop a series of ‘points to consider’ to assist clinicians at the earliest stages of evaluation and diagnosis, management, and monitoring of patients with HLH/MAS in order to improve patient outcomes.

Methods A working group of adult and pediatric rheumatologists (14), hematologist/oncologists (4), immunologists (2), infectious disease specialists (2), intensivists (3), allied health care professionals (1), and patients/parents (2) formulated relevant research questions for a systematic literature review (SLR). We then used the SLR results, Delphi questionnaires, and consensus methodology to devise and refine overarching and specific ‘points to consider’ statements.

Results The group arrived at six overarching statements and 24 specific points-to-consider relevant to early decision-making in diagnostics, initial management, and monitoring of HLH/MAS. Major themes included the a) need for prompt recognition, evaluation, and management of underlying triggers and conditions, b) multi-disciplinary/expert input, and c) early, tailored intervention with the goals of halting disease progression and preventing life- and organ-threatening immunopathology

Conclusion These 2022 EULAR/ACR Points to Consider provide guidance on the initial evaluation, management, and monitoring of patients during the initial consideration of HLH/MAS.

Disclosure of Interests Bita Shakoory: None declared, Ashley Geerlinks: None declared, Marta Wilejto: None declared, Kate Kernan: None declared, Erkan Demirkaya: None declared, Angelo Ravelli: None declared, Rashmi Sinha: None declared, Raphaela goldbach-mansky Grant/research support from: SOBI, Novartis, Regneneron, IFM, Lilly, Pfizer, Fabrizio De Benedetti Consultant of: abbvie, sobi, novimmune, novartis, roche, sanofi, Grant/research support from: sobi novimmune novartis roche sanofi, Rebecca Marsh: None declared, Scott Canna Consultant of: Simcha Therapeutics, Grant/research support from: Immvention therapeutics, AB2Bio Ltd, Novartis

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