摘要
目的:探讨1例原发性肾上腺NK/T细胞淋巴瘤(primary adrenal natural killer/T-cell lymphoma,PANKTCL)患者的临床特点及诊疗方案,加强对该罕见类型淋巴瘤的认识。方法:回顾性分析本院收治的1例PANKTCL患者的临床表现、诊疗过程及预后。结果:结合病理学、影像学及骨髓检查等,患者诊断为PANKTCL(CA分期,II期;PINK-E评分3分,高危组)。治疗上行“P-GemOx+VP-16”(吉西他滨1 g/m^(2) d 1+奥沙利铂100 mg/m^(2) d 1+依托泊苷60 mg/m^(2) d 2-4+培门冬酶3750 IU d 5)方案6个周期治疗,4个周期评估达完全缓解。化疗结束后予以信迪利单抗维持治疗。完全缓解后8个月患者出现疾病复发,共行4个疗程化学治疗,期间发生噬血细胞综合征,1个月后患者疾病进展死亡。结论:PANKTCL罕见,易复发且预后差,“P-GemOx+VP-16”联合信迪利单抗方案治疗有助于改善非上呼吸道消化道NK/T细胞淋巴瘤患者的生存预后。
Objective:To investigate the clinical characteristics,diagnosis,and treatment of one patient with primary adrenal natural killer/T-cell lymphoma(PANKTCL),and to strengthen the understanding of this rare type of lymphoma.Methods:The clinical manifestations,diagnosis and treatment process,and prognosis of the patient admitted in our hospital were retrospectively analyzed.Results:Combined with pathology,imaging,bone marrow examination,etc,the patient was diagnosed with PANKTCL(CA stage,stage II;PINK-E score 3,high-risk group).Six cycles of“P-GemOx+VP-16”regimen(gemcitabine 1 g/m^(2) d1+oxaliplatin 100 mg/m^(2) d 1+etoposide 60 mg/m^(2) d 2-4+polyethylene glycol conjugated asparaginase 3750 IU d 5)was performed,and complete response was assessed in 4 cycles.Maintenance therapy with sintilimab was administered after the completion of chemotherapy.Eight months after the complete response,the patient experienced disease recurrence and underwent a total of four courses of chemotherapy,during which hemophagocytic syndrome occurred.The patient died of disease progression 1 month later.Conclusion:PANKTCL is rare,relapses easily,and has a worse prognosis.The choice of the“P-GemOx+VP-16”regimen combined with sintilimab help to improve the survival prognosis of patient with non-upper aerodigestive tract natural killer/T-cell lymphoma.
作者
季新悦
沈大鹏
杨玉琼
魏元凤
黄曦
刘琼
喻丹宁
郭于鑫
何合胜
JI Xin-Yue;SHENG Da-Peng;YANG Yu-Qiong;WEI Yuan-Feng;HUANG Xi;LIU Qiong;YU Dan-Ning;GUO Yu-Xin;HE He-Sheng(Department of Hematology,Yijishan Hospital of Wannan Medical College,Wuhu 241000,Anhui Province,China)
出处
《中国实验血液学杂志》
CAS
CSCD
北大核心
2023年第2期396-402,共7页
Journal of Experimental Hematology
基金
芜湖市科技成果转化计划(2021cg27)
安徽省质量工程项目(2020jyxm2104)
吴阶平医学基金会(320.6750.2020-10-43)。
