Yang Chun Li, Chen Xi, Zhou Hui Jie, Wu Wan Chun, Zou Li Qun
State Key Laboratory of Biotherapy and Cancer Center, West China Hospital, Sichuan University, Chengdu, China.
Division of Radiotherapy, Cancer Center, West China Hospital, Sichuan University, Chengdu, China.
J Hematol. 2024 Apr;13(1-2):46-51. doi: 10.14740/jh1242. Epub 2024 Apr 9.
Extranodal natural killer/T-cell lymphoma-associated hemophagocytic lymphohistiocytosis (ENKTCL-LAHS) is a rare disease with poor prognosis. Currently, there are no well-established treatments for LAHS. Almost 50% of patients experience relapsed or refractory disease to anti-hemophagocytic lymphohistiocytosis (HLH) treatment, and the regimen for salvage therapy is limited. We report a case of ENKTCL-LAHS that was successfully treated with a programmed cell death ligand 1 (PD-L1) antibody (sugemalimab) alone and provide a literature review on existing ENKTCL-LAHS treatment options. A 31-year-old man with relapsed ENKTCL complicated by HLH was admitted to our hospital. Following the administration of the PD-L1 antibody sugemalimab, fever was resolved, Epstein-Barr virus (EBV) DNA copy number was negative, and HLH-related blood biochemical markers were decreased in the patient. Consequently, the patient achieved complete remission with a progression-free time (PFS) of 44 months. The prognosis of ENKTCL-LAHS is extremely poor, and the clinical treatment of ENKTCL-HLH is challenging. No previous reports exist regarding the use of PD-L1 antibodies in ENKTCL-LAHS treatment. This study is the first to report a patient with ENKTCL-LAHS treated with the PD-L1 antibody alone, who achieved a long PFS of 44 months. Our results suggest the effectiveness and safety of sugemalimab in the treatment of ENKTCL-LAHS; however, more clinical cases are required for validation. The PD-L1 antibody presents a novel treatment option for patients with ENKTCL-LAHS and warrants further clinical promotion.
结外自然杀伤/T细胞淋巴瘤相关噬血细胞性淋巴组织细胞增生症（ENKTCL-LAHS）是一种预后较差的罕见疾病。目前，对于LAHS尚无成熟的治疗方法。近50%的患者对抗噬血细胞性淋巴组织细胞增生症（HLH）治疗出现复发或难治性疾病，挽救治疗方案有限。我们报告1例仅用程序性细胞死亡配体1（PD-L1）抗体（苏金单抗）成功治疗的ENKTCL-LAHS病例，并对现有的ENKTCL-LAHS治疗选择进行文献综述。一名31岁复发的ENKTCL合并HLH的男性患者入住我院。给予PD-L1抗体苏金单抗后，患者发热消退，EB病毒（EBV）DNA拷贝数转阴，HLH相关血液生化指标下降。因此，患者实现完全缓解，无进展生存期（PFS）为44个月。ENKTCL-LAHS的预后极差，ENKTCL-HLH的临床治疗具有挑战性。既往尚无关于在ENKTCL-LAHS治疗中使用PD-L1抗体的报道。本研究首次报告1例仅用PD-L1抗体治疗的ENKTCL-LAHS患者，其PFS长达44个月。我们结果提示苏金单抗治疗ENKTCL-LAHS有效且安全；然而，需要更多临床病例进行验证。PD-L1抗体为ENKTCL-LAHS患者提供了一种新的治疗选择，值得进一步临床推广。
