Lipsitt Amanda E, Hung Jaclyn Y, Langevin Anne-Marie
Department of Pediatrics Hematology-Oncology, University of Texas Health San Antonio, 7703 Floyd Curl Drive, Mail Code 7810, San Antonio, TX, 78229, USA.
J Med Case Rep. 2021 Mar 5;15(1):102. doi: 10.1186/s13256-021-02740-6.
Extranodal NK/T cell lymphoma (ENKTL) is an aggressive form of Epstein-Barr virus (EBV)-associated non-Hodgkin's lymphoma which historically has a poor prognosis. When relapse occurs, particularly in the cerebral nervous system (CNS), survival is rare. The immune checkpoint pathway family of proteins is highly expressed in many human tumors, especially in EBV-related malignancies. To the best of our knowledge, there are no reports of immune checkpoint inhibitors used either alone or in combination for the treatment of ENTKL CNS relapse, yet there are promising results in metastatic CNS involvement of other malignancies.
This is the case of a 29-year-old Hispanic male with ENKTL who was treated at first relapse with 24 doses of the programmed death-ligand 1 (PD-L1) immune checkpoint inhibitor, atezolizumab, over a 17-month period. He remained in remission for 18 months until he experienced an isolated CNS relapse and on-going evidence of chronic EBV infection. Salvage therapy was provided as a combination of triple intrathecal (TIT) chemotherapy, radiation, and atezolizumab. He continues on maintenance atezolizumab and remains alive 1-year post CNS relapse.
The results from this case suggest that atezolizumab should be considered as part of the treatment regimen for relapsed ENKTL. They also demonstrate the benefit of using atezolizumab in combination with TIT chemotherapy and radiation as a viable treatment option for ENKTL CNS relapse and indicate that atezolizumab is an option for long-term maintenance therapy for patients with ENKTL.
结外NK/T细胞淋巴瘤（ENKTL）是一种侵袭性的与爱泼斯坦-巴尔病毒（EBV）相关的非霍奇金淋巴瘤，历来预后较差。当复发发生时，尤其是在中枢神经系统（CNS），存活情况罕见。免疫检查点蛋白通路家族在许多人类肿瘤中高度表达，尤其是在EBV相关的恶性肿瘤中。据我们所知，尚无关于单独或联合使用免疫检查点抑制剂治疗ENKTL中枢神经系统复发的报道，不过在其他恶性肿瘤的转移性中枢神经系统受累方面有令人鼓舞的结果。
这是一名29岁的西班牙裔男性ENKTL患者，首次复发时在17个月内接受了24剂程序性死亡配体1（PD-L1）免疫检查点抑制剂阿特珠单抗的治疗。他缓解了18个月，直到出现孤立的中枢神经系统复发以及持续的慢性EBV感染证据。挽救性治疗采用鞘内三联（TIT）化疗、放疗和阿特珠单抗联合的方式。他继续接受阿特珠单抗维持治疗，中枢神经系统复发后1年仍然存活。
该病例结果表明，阿特珠单抗应被视为复发ENKTL治疗方案的一部分。它们还证明了将阿特珠单抗与TIT化疗和放疗联合使用作为ENKTL中枢神经系统复发的可行治疗选择的益处，并表明阿特珠单抗是ENKTL患者长期维持治疗的一种选择。
