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科普贴:外周T细胞淋巴瘤(PTCL)

2021-08-06 网络 网络

PTCL概况

PTCL概况

外周T细胞淋巴瘤(peripheral T cell lymphomas, PTCLs)是一组起源于胸腺后T淋巴细胞或成熟NK细胞异质性疾病。在欧美国家约占非霍奇金淋巴瘤的 10-15%左右,而我国为21.4%左右[ 1-2]。相较于其它类型淋巴瘤,PTCLs的发生率仍较低,这使其诊断与治疗非常具有挑战性。其中外周T细胞淋巴瘤非特指型(Peripheral T Cell Lymphoma, Not Otherwise Specified, PTCL-NOS)预后评分系统包括IPI和PTCL-NOS预后指数(Prognostic index for PTCL-NOS, PIT)[3]

预后评估

国际预后评分(International Prognostic Index, IPI)用于PTCLs的风险评估,与总生存率(Overall Survival, OS)相关,分值越高,预后越差。

Ann-Arbor分期

淋巴瘤的分期系统采用Ann-Arbor (Cotswolds修订)分期系统:

Ⅰ期:侵及一个淋巴结区(Ⅰ),或侵及一个单一的淋巴结外器官或部位(ⅠE);

Ⅱ期:在横膈的一侧,侵及二个或更多的淋巴结区(Ⅱ)或外加局限侵犯一个结外器官或部位(ⅡE);

Ⅲ期:受侵犯的淋巴结区在横膈的两侧(Ⅲ)或外加局限侵犯一个结外器官或部位(ⅢE)或脾(ⅢS)或 二者均有(ⅢES);

Ⅳ期:弥漫性或播散性侵犯一个或更多的结外器官,同时伴有或不伴有淋巴结侵犯。[3]

PTCL常见亚型

外周T细胞淋巴瘤不是一种疾病,而是一组疾病,2016年世界卫生组织(World Health Organization, WHO)分类系统[4]将PTCLs划分为4个类别:淋巴结型、结外型、白血病型和皮肤型,总计25个以上亚型(具体参见文末2016WHO分类T细胞肿瘤)。PTCLs在男性和老年人中更为常见,尽管临床表现因具体类型而有不同,但PTCLs预后通常较差,在某些PTCL 亚型中,5年生存率可能低至7%。复发或难于治疗的患者往往处于预后差的高风险之下[5]。

目前临床比较关注的几个常见的PTCLs亚型有:

1

外周T细胞淋巴瘤非特指型(Peripheral T Cell Lymphoma, Not Otherwise Specified, PTCL-NOS)是最常见的组织学亚型。PTCL-NOS并不是单一疾病,而是一组不能划入其他PTCLs的异质性疾病。PTCL-NOS包括三种亚型,分别过表达GATA3,TBX21和细胞毒基因。

2

血管免疫母细胞T细胞淋巴瘤(Angioimmunoblastic T-Cell Lymphoma, AITL)。AITL发生与 EB病毒(Epstein-Barrvirus,EBV)、人类疱疹病毒(human herpesvirus,HHV)、 人类免疫缺陷病毒(human immunodeficiency virus,HIV)、细菌和真菌感染有关,且这些病毒感染可能与免疫失调有关。

3

间变性大细胞淋巴瘤(Anaplastic Large-Cell Lymphoma, ALCL),ALCL又可以分为系统性间变大、皮肤原发间变大及乳房植入物相关性间变大。系统性间变大可分为ALK阳性和ALK阴性。ALCL的主要特点是肿瘤细胞呈现高度异质性且几乎全部表达CD30抗原,免疫表型可以是T细胞表达或者非T非B细胞表达。

PTCLs主要亚型发病率占比因地区而异,原发于淋巴结的PTCLs以欧洲最常见,其中PTCLs-NOS占34%,AITL占28%,ALK+ALCL占6%,ALK-ALCL占9%,而在亚洲原发于淋巴结外的PTCLs更常见,日本以人类T淋巴细胞病毒-1型(Human T lymphocyte virus I, HTLV-1)相关的成人T细胞白血病/淋巴瘤(Adult T cell leukemia/lymphoma, ATLL)为主,而亚洲非日本国家以结外自然杀伤或T细胞淋巴瘤(Extranodel nature killer or T cell lymphoma, ENKTCL)为主,在北欧,由于人类白细胞抗原单倍体相关腹部疾病发生率较高,所以肠道病相关T细胞淋巴瘤(Enteropathy-associated T cell lymphoma, EATL)发生更频繁[5]。

2016版WHO T细胞肿瘤分类

(※:与2008 WHO分类的不同之处;红体字:临时分类)[6]

参考文献

[1]James O. Armitage . The aggressive peripheral T-cell lymphomas: 2017, Am J Hematol. 2017;92:706–715.

[2]李小秋等.中国淋巴瘤亚型分布:国内多中心病例10002 例分析.诊断学理论与实际.2012;11(2):111-115

[3]淋巴瘤诊疗规范(2020年版)

[4]中国临床肿瘤学会(CSCO)淋巴瘤诊疗指南2021版

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