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KMID : 0387820220290020092
Clinical Pediatric Hematology-Oncology
2022 Volume.29 No. 2 p.92 ~ p.96
Anakinra to Mitigate Hemophagocytic Lymphohistiocytosis-Like Toxicity Following Chimeric Antigen Receptor T-cell Therapy in Pediatric B-cell ALL
Lee Na-Yoon

Jo Sue-Jung
Yoo Jae-Won
Kim Seong-Koo
Lee Jae-Wook
Chung Nack-Gyun
Cho Bin
Abstract
Hemophagocytic lymphohistiocytosis-like toxicity following chimeric antigen re-ceptor (CAR)-T cell therapy (carHLH) is a rare and fulminant complication. Currently, there are neither well-established diagnostic criteria nor optimal treatment option for carHLH. Given the similarities of hyperinflammatory process and cytokine profiles between cytokine release syndrome (CRS) and carHLH, tocilizumab and cortico-steroids are the suggested front-line treatment options for both conditions. However, when carHLH is refractory to front-line treatment, alternative or adjunctive agents should be introduced to ameliorate ongoing hyperinflammation. Anakinra, a re-combinant interleukin (IL)-1 receptor antagonist, has shown promising results in the management of carHLH. Although prospective trials are limited, the use of anakinra for severe CRS and carHLH has been increasing. In this case report, we present two cases of carHLH to discuss its characteristic clinical features, diagnostic criteria, and treatment option. In addition, we also highlight the clinical efficacy of anakinra for managing carHLH which was refractory to tocilizumab and dexamethasone.
KEYWORD
Lymphohistiocytosis, Hemophagocytic, CD19-specific chimeric antigen receptor, Interleukin 1 receptor antagonist protein
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