Department of General Internal Medicine, Medical College of Wisconsin, Milwaukee, WI 53226, USA.
Department of Internal Medicine, Baylor College of Medicine, Houston, TX 77030, USA.
Med Sci (Basel). 2023 Oct 12;11(4):67. doi: 10.3390/medsci11040067.
Chimeric antigen receptor T-cells (CAR-T) are now a standard approach for treating relapsed/refractory B-cell lymphomas. Immune effector cell-associated hemophagocytic lymphohistiocytosis-like syndrome (IEC-HS) is a newly described entity that can manifest following CAR-T. Bone marrow (BM) aplasia is an uncommon manifestation of IEC-HS reported after CAR-T-cell therapy and is defined as the reduction or absence of hematopoietic progenitor cells resulting in severe pancytopenia. We describe the case of a 44-year-old female with relapsed/refractory Burkitt lymphoma (BL) who received treatment with lisocabtagene maraleucel with her post-CAR-T course complicated by cytokine release syndrome (CRS) and IEC-HS ultimately leading to persistent BM aplasia. She underwent a rescue allogeneic stem cell transplant but ultimately succumbed to progressive disease. IEC-HS is an increasingly recognized complication that occurs after CAR-T treatments that can result in aplasia, a dangerous complication with serious sequelae including infection, transfusion dependence, and high risk for hemorrhage. The underlying mechanism is poorly understood, and further studies are needed to understand how to treat it better.
嵌合抗原受体 T 细胞（CAR-T）现已成为治疗复发/难治性 B 细胞淋巴瘤的标准方法。免疫效应细胞相关噬血细胞性淋巴组织细胞增生症样综合征（IEC-HS）是一种新描述的实体，可在 CAR-T 后出现。骨髓（BM）再生不良是 CAR-T 细胞治疗后报告的 IEC-HS 的一种罕见表现，定义为造血祖细胞减少或缺乏导致严重全血细胞减少。我们描述了一例 44 岁女性复发性/难治性伯基特淋巴瘤（BL）患者，接受了 lisocabtagene maraleucel 治疗，其 CAR-T 后病程中出现细胞因子释放综合征（CRS）和 IEC-HS，最终导致持续性 BM 再生不良。她接受了挽救性异基因干细胞移植，但最终因疾病进展而死亡。IEC-HS 是 CAR-T 治疗后越来越被认识到的一种并发症，可导致再生不良，这是一种危险的并发症，严重后果包括感染、输血依赖和出血风险增加。其潜在机制尚不清楚，需要进一步研究以了解如何更好地治疗它。
