Cancer Center, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Eur Rev Med Pharmacol Sci. 2023 May;27(10):4570-4577. doi: 10.26355/eurrev_202305_32463.
The prognosis of natural killer/T cell lymphoma (NKTCL) with multifocal small intestine involvement complicated by intestinal perforation is extremely poor. There is no evidence-based treatment strategy for this intractable condition.
A 30-year-old male was admitted to our hospital in April 2017 and presented with recurrent fever for three months and multiple painless subcutaneous nodules in the abdominal wall. An excision biopsy of the subcutaneous nodules in the abdominal wall revealed NKTCL. The patient was diagnosed with stage IVB NKTCL with skin and multifocal small intestinal involvement according to the imaging results. The first intestinal perforation occurred due to tumor infiltration before the initial treatment. The second intestinal perforation occurred after receiving two cycles of chemotherapy with a modified SMILE regimen. The histone deacetylase inhibitor (HDACi) chidamide was administered as a single-agent therapy after recovery from the second intestinal perforation. Complete remission was achieved. Unfortunately, five months later, the patient was confirmed to have relapsed and received the salvage chemotherapy. The patient suffered from disease progression again after the fourth cycle of chemotherapy. At this point, from May 29, 2018, the patient started to receive injections of the anti-programmed death 1 (PD-1) antibody camrelizumab as a salvage treatment. Two months after the initial anti-PD-1 antibody camrelizumab injection, the response was partial remission. Disease progression was confirmed in March 2021, with a progression-free survival time of 34 months.
NKTCL patients with multifocal small intestine involvement have a high risk of intestinal perforation. The possible etiologies of bowel perforation include tumor infiltration, tumor necrosis in response to therapy, and acute inflammation. The anti-PD-1 antibody camrelizumab may be a new candidate agent for treating this type of intractable NKTCL. Further observations are necessary to identify the efficacy and safety of new agents in the future.
伴有小肠多处累及并穿孔的自然杀伤(NK)/T 细胞淋巴瘤(NKTCL)的预后极差,针对这种棘手的情况,目前尚无循证治疗策略。
一名 30 岁男性于 2017 年 4 月因反复发热 3 个月,伴腹壁多发无痛性皮下结节就诊于我院。腹壁皮下结节切除活检结果提示 NKTCL。根据影像学结果,该患者诊断为 IVB 期 NKTCL,伴皮肤和多灶性小肠累及。在初始治疗前,肿瘤浸润导致首次发生肠穿孔。在接受两周期改良 SMILE 方案化疗后,发生第二次肠穿孔。第二次肠穿孔恢复后,给予组蛋白去乙酰化酶抑制剂(HDACi)西达本胺单药治疗,达到完全缓解。不幸的是,5 个月后,患者疾病复发,接受挽救性化疗。第四次化疗后,患者再次出现疾病进展。此时,自 2018 年 5 月 29 日起,患者开始接受抗程序性死亡受体 1(PD-1)抗体卡瑞利珠单抗的挽救治疗。首次注射抗 PD-1 抗体卡瑞利珠单抗 2 个月后,疗效评估为部分缓解。2021 年 3 月,疾病进展再次被确认,无进展生存期为 34 个月。
多灶性小肠累及的 NKTCL 患者发生肠穿孔的风险较高。肠穿孔的可能病因包括肿瘤浸润、肿瘤治疗后坏死和急性炎症。抗 PD-1 抗体卡瑞利珠单抗可能是治疗这种难治性 NKTCL 的新候选药物。未来还需要进一步观察,以确定新药物的疗效和安全性。