Cellular Therapies and Transfusion Medicine, Careggi University Hospital, Florence, Italy.
Department of Haematology, Careggi University Hospital, Florence, Italy.
Transpl Immunol. 2022 Dec;75:101719. doi: 10.1016/j.trim.2022.101719. Epub 2022 Sep 16.
Secondary haemophagocytic lymphohistiocytosis (sHLH) is a life-threatening disorder described in the setting of infections, neoplastic and immune dysregulations. Recently, sHLH has been reported following chimeric antigen receptor T-cell (CAR-T) therapy as a severe manifestation of cytokine release syndrome (CRS) which generally occurs during the early phase after a CAR-T infusion. CAR-T therapy for both relapse/refractory acute lymphoblastic B-cell leukaemia (B-ALL) and non-Hodgkin lymphoma, (diffuse large B-cell lymphoma (DLBCL) and primary mediastinal B-cell lymphoma (PMBCL)), has been approved by FDA and EMA as a third line treatment. CRS is on-target off-tumour side effect of CAR-T therapy which results in an acute state of hyperinflammation due to both tumour lysis and the proliferation of CAR-T cells. Its clinical presentation has a wide spectrum of severity, in the worst case it could rapidly lead to a multiorgan failure and progress to a fatal sHLH. Here, we present a late occurrence of sHLH after CAR-T treatment.
继发性噬血细胞性淋巴组织细胞增生症(sHLH)是一种危及生命的疾病,可发生于感染、肿瘤和免疫失调等情况下。最近,嵌合抗原受体 T 细胞(CAR-T)治疗后也有 sHLH 的报道,其是细胞因子释放综合征(CRS)的严重表现,通常发生在 CAR-T 输注后的早期阶段。FDA 和 EMA 已批准 CAR-T 治疗复发/难治性急性 B 淋巴细胞白血病(B-ALL)和非霍奇金淋巴瘤(弥漫性大 B 细胞淋巴瘤(DLBCL)和原发性纵隔 B 细胞淋巴瘤(PMBCL))作为三线治疗。CRS 是 CAR-T 治疗的针对肿瘤的脱靶副作用,由于肿瘤溶解和 CAR-T 细胞的增殖,导致急性炎症状态。其临床表现具有广泛的严重程度,在最坏的情况下,它可能会迅速导致多器官衰竭,并进展为致命的 sHLH。在这里,我们报告了一例 CAR-T 治疗后 sHLH 的迟发病例。
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