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Hyperinflammatory syndrome resembling haemophagocytic lymphohistiocytosis following axicabtagene ciloleucel and brexucabtagene autoleucel

Timothy J. Porter, Ana Lazarevic, Jamie E. Ziggas, Ephraim J. Fuchs, Kiryoung Kim, Helen Byrnes, Leo Luznik, Javier Bolaños‐Meade, Syed Abbas Ali, Nirali N. Shah, Nina D. Wagner‐Johnston, Tania Jain

2022British Journal of Haematology25 citationsDOIOpen Access PDF

Abstract

Haemophagocytic lymphohistiocytosis-like toxicity following chimeric antigen receptor T cells (CAR-HLH) is being increasingly recognized, while published data are limited and criteria for recognition are elusive. We describe three patients who developed CAR-HLH after infusion of brexucabtagene autoleucel (n = 2) or axicabtagene ciloleucel (n = 1). All three patients presented following cytokine release syndrome, with fever, recurrent or worsening cytopenias, hyperferritinaemia, elevated soluble interleukin (IL)-2 receptor, hypofibrinogenaemia, hypertriglyceridaemia, elevated liver transaminases, and decreasing C-reactive protein and IL-6. Clinical improvement following treatment with anakinra (n = 2) and ruxolitinib (n = 1) was observed. Our report offers an opportunity for prompt recognition and initiation of potentially life-saving treatment for CAR-HLH.

Topics & Concepts

MedicineHemophagocytic lymphohistiocytosisAnakinraImmunologyCytokine release syndromeChimeric antigen receptorInternal medicineGastroenterologyImmunotherapyImmune systemDiseaseCAR-T cell therapy researchAutoimmune and Inflammatory Disorders ResearchImmune Cell Function and Interaction