单倍型异基因造血干细胞移植治疗难治急性型成人T细胞白血病-淋巴瘤1例并文献复习  

Haploidentical allogeneic hematopoietic stem cell transplantation for the acute refractory adult T cell leukemia-lymphoma:A case report and literature review

作　　者：朱佳琳 唐博[1] 尹玥[1] 许蔚林[1] 王倩[1] 欧晋平[1] 王文生[1] 董玉君[1] ZHU Jialin;TANG Bo;YIN Yue;XU Weilin;WANG Qian;OU Jinping;WANG Wensheng;DONG Yujun(Department of Hematology,Peking University First Hospital,Beijing 100034,China)

机构地区：[1]北京大学第一医院血液内科,北京100034

出　　处：《现代肿瘤医学》2022年第23期4294-4298,共5页Journal of Modern Oncology

基　　金：国家自然科学基金(编号:8180010805)。

摘　　要：目的:探讨成人T细胞白血病-淋巴瘤(adult T cell leukemia-lymphoma,ATL)的临床特征及治疗。方法:回顾分析我院1例ATL的临床病理特征、治疗转归,并进行相关文献复习。结果:患者女性,44岁,祖籍福建,以皮疹、腹胀及水肿为首发表现,影像学检查提示全身多发代谢增高肿大淋巴结、脾大,腋窝淋巴结、腹壁皮肤活检提示外周T细胞淋巴瘤;经预治疗及多次化疗后效果欠佳,行HTLV I-RNA检测为阳性,诊断ATL急性型明确,后选择其子作为供者行单倍型异基因造血干细胞移植,移植后随访15个月至2022年04月仍存活。结论:由于北方地区HTLV-1感染率较低,遇到化疗效果欠佳的外周T细胞淋巴瘤时需考虑到ATL可能,尽早行相关病毒学检测以尽快明确诊断。单倍型异基因造血干细胞移植可以为ATL患者改善长期预后带来希望。Objective:To investigate adult T cell leukemia-lymphoma's(ATL)clinical characteristics and treatment.Methods:The clinicopathological features,treatment and outcome of a case diagnosed with the acute subtype of ATL in our hospital were retrospectively analyzed and a review of relevant literature.Results:A 44 years old female from Fujian province,presented with rash,abdominal distension and edema.Imaging examination suggested multiple enlarged lymph nodes with uptake metabolism and splenomegaly.Axillary lymph nodes and skin biopsy of the abdominal wall suggested peripheral T-cell lymphoma.After induction therapy and several courses of chemotherapies,the response was not good.After the detection of HTLV I-RNA,the diagnosis of acute subtype of ATL was established.Later,his son was selected as the donor for haploidentical allogeneic hematopoietic stem cell transplantation.The patient was still alive after transplantation for 15 months of follow-up until April 2022.Conclusion:Although the infection rate of HTLV-1 in northern China is low,the possibility of ATL should be considered in the case of peripheral T-cell lymphoma with poor chemotherapy response,and the early detection of the related virology should be carried out to confirm the diagnosis as soon as possible.Haploidentical allogeneic hematopoietic stem cell transplantation(HSCT)offers hope of improving aggressive ATL patients'long-term prognosis.

关 键 词：成人T细胞白血病-淋巴瘤 HTLV-1 单倍型异基因造血干细胞移植 

分 类 号：R733.7[医药卫生—肿瘤]