肝脾T细胞淋巴瘤的研究进展  

Recent advances in hepatosplenic T-cell lymphoma

作　　者：马银娟 王莹 杨柯 潘耀柱 王璇 MA Yinjuan;WANG Ying;YANG Ke;PAN Yaozhu;WANG Xuan(The 940th Hospital of the PLA Joint Service Support Force,Gansu Lanzhou 730050,China;Graduate School of Ningxia Medical University,Ningxia Yinchuan 750004,China.)

机构地区：[1]中国人民解放军联勤保障部队第九四〇医院,甘肃兰州730050 [2]宁夏医科大学研究生院,宁夏银川750004

出　　处：《现代肿瘤医学》2021年第13期2343-2347,共5页Journal of Modern Oncology

基　　金：国家自然科学基金(编号:81372132)。

摘　　要：肝脾T细胞淋巴瘤(HSTCL)是一种罕见的特殊类型的侵袭性外周T细胞淋巴瘤,主要表现为发热、盗汗、体重减轻及腹痛,多累及脾、肝和骨髓,通常无淋巴结肿大。因HSTCL具有异质性及侵袭性,其预后极差。目前有关HSTCL治疗的文献多数出自个案报告,缺乏随机前瞻性试验,因此暂无统一的一线疗法,大剂量化疗诱导联合异基因造血干细胞移植的方案目前较受认可。由于此病罕见且研究相对较少,本文回顾相关文献将HSTCL的病因、发病机制、临床表现及诊断治疗做一综述。Hepatosplenic T-cell lymphoma(HSTCL)is a rare special type of aggressive peripheral T cell lymphoma.The main symptoms of the patients are fever,night sweats,weight loss,and abdominal pain,and extranodal lymphatic tissue infiltration such as the spleen,liver,and bone marrow,usually without lymph node enlargement.Due to the heterogeneity and aggressiveness of HSTCL,its prognosis is extremely poor.At present,most of the literature of HSTCL therapy experience is mostly based on case reports,and randomized prospective trials are limited by its rarity.Therefore,there is no unified first-line therapy at present,and the regimen of high-dose inducing chemotherapy combined with allogeneic hematopoietic stem cell transplantation is currently well accepted now.And due to the rarity of HSTCL and relatively few studies,the etiology,pathogenesis,clinical manifestations,diagnosis and treatment of HSTCL are reviewed in this paper.

关 键 词：肝脾T细胞淋巴瘤 肝脾γδ-T细胞淋巴瘤 肝脾αβ-T细胞淋巴瘤 造血干细胞移植 

分 类 号：R735.7[医药卫生—肿瘤]