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CAR T cell therapy-associated cytokine release syndrome

Disease definition

A rare systemic condition affecting patients undergoing chimeric antigen receptor (CAR) T-cell therapy and characterized by a systemic inflammatory response due to massive activation of leukocytes with subsequent cytokine release. It can present with a variety of signs and symptoms ranging from mild, flu-like symptoms (such as fever, fatigue, headache, rash, arthralgia, and myalgia) to severe life-threatening manifestations including vascular leakage, disseminated intravascular coagulation, shock, and multiple organ failure. Respiratory manifestations are common and range from cough and tachypnea to acute respiratory distress syndrome (ARDS).

ORPHA:542323

Classification level: Disorder
  • Synonym(s):
    • CAR T cell therapy-associated CRS
    • Chimeric antigen receptor-T cell therapy-associated cytokine release syndrome
  • Prevalence: Unknown
  • Inheritance: -
  • Age of onset: Adult
  • ICD-10: D89.8
  • OMIM: -
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -
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