Topics:
Pediatric Oncology • Medical Oncology • Rheumatology • CAR-T • Hematology • Pediatric Hematology • HLH • Pediatric Rheumatology • Multispecialty Spotlight
How do you approach diagnosis and treatment of HLH/MAS following CAR T-cell therapy?
How is this entity distinct from other secondary HLH entities?
Answer from: at Academic Institution
I maintain that immune effector cell associated hyperinflammatory syndrome is NOT HLH. Most patients post CAR-T cell therapy fulfill the criteria for HLH even if they don’t have hyperinflammatory syndrome, so it makes diagnosis very challenging. Many patients with this “HLH-like” h...
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Answer from: at Academic Institution
In pediatrics, I have always believed that secondary HLH/MAS is the most severe end of the spectrum of hyperinflammatory syndromes and most of the manifestations are due to the release of proinflammatory cytokines (although increased levels of anti-inflammatory IL10 is usually seen as well). The cyt...