文章：

免疫效应细胞相关HLH样综合征：文献综述——一种日益被认识的疾病实体

Immune Effector Cell-Associated HLH-like Syndrome: A Review of the Literature of an Increasingly Recognized Entity

原文发布日期：26 October 2023

DOI: 10.3390/cancers15215149

类型: Article

开放获取: 是

英文摘要：

Since CAR-T cell therapy was initially approved in 2017, its use has become more prevalent and so have its side effects. CAR-T-related HLH, also named immune effector cell-associated HLH-like syndrome (IEC-HS), is a rare but fatal toxicity if not recognized promptly. We conducted a review of the literature in order to understand the prevalence of IEC-HS as well as clarify the evolution of the diagnostic criteria and treatment recommendations. IEC-HS occurrence varies between CAR-T cell products and the type of malignancy treated. Diagnosis can be challenging as there are no standardized diagnostic criteria, and its clinical features can overlap with cytokine release syndrome and active hematological disease. Suggested treatment strategies have been extrapolated from prior experience in HLH and include anakinra, corticosteroids and ruxolitinib. IEC-HS is a potentially fatal toxicity associated with CAR-T cell therapy. Early recognition with reliable diagnostic criteria and prompt implementation of treatment specific to IEC-HS is imperative for improving patient outcomes.

摘要翻译： 

自2017年CAR-T细胞疗法首次获批以来，其应用日益广泛，相关副作用也随之增多。CAR-T相关噬血细胞性淋巴组织细胞增多症（HLH），亦称免疫效应细胞相关HLH样综合征（IEC-HS），是一种罕见但若未能及时识别则具有致命风险的毒性反应。本文通过文献综述，旨在明确IEC-HS的流行病学特征，梳理诊断标准的演变过程及治疗建议的更新脉络。IEC-HS的发生率因CAR-T细胞产品类型及所治疗恶性肿瘤种类而异。由于缺乏标准化诊断标准，且其临床特征可能与细胞因子释放综合征及活动性血液系统疾病相互重叠，诊断工作面临挑战。现有治疗策略主要借鉴HLH的既往经验，包括阿那白滞素、皮质类固醇和鲁索替尼等药物的应用。IEC-HS是CAR-T细胞疗法潜在的致命毒性反应，建立可靠的诊断标准以实现早期识别，并及时实施针对IEC-HS的特异性治疗，对改善患者预后至关重要。

原文链接：

Immune Effector Cell-Associated HLH-like Syndrome: A Review of the Literature of an Increasingly Recognized Entity