淋巴母细胞性T细胞淋巴瘤与EB病毒的关系

目的：探讨淋巴母细胞性Ｔ细胞淋巴瘤与ＥＢ病毒的关系。方法：收集９例淋巴母细胞性Ｔ细胞淋巴瘤进行ＥＢ病毒检测，用免疫组织化学ＡＢＣ法证实其肿瘤细胞本质；用聚合酶链反应检测ＥＢ病毒特征性ＤＮＡ序列（ＥＢＶ ＤＮＡ）；用ＲＮＡ原位杂交法检测ＥＢ病毒编码的ＲＮＡ（ＥＢＥＲ１／２）。结果：９例淋巴母细胞性Ｔ细胞淋巴瘤，ＥＢＶ ＤＮＡ阳性７例（７７．８％），ＥＢＥＲ１／２阳性６例（６６．７％）。结论：淋巴母细     

非特指外周T细胞淋巴瘤中PTEN的改变

目的观察非特指外周T细胞淋巴瘤(peripheral T cell lymphoma,not otherwise specified,PTCL-NOS)中抑癌基因(phos-phatase and tensin homolog deleted on chromosome ten,PTEN)的改变情况,探讨其与肿瘤生物学行为的关系,为阐明PTCL-NOS的发生、发展机制提供科学依据。方法应用间期双色荧光原位杂交(fluorescence in situ hybridization,FISH)技术检测36例PTCL-NOS石蜡包埋组织中PTEN基因的改变情况,分析其改变与各临床参数的关系。结果 36例PTCL-NOS中8例出现PTEN杂合性缺失(loss of heterozygosity,LOH);Kaplan-Meier生存分析显示该基因异常组较正常组生存期明显缩短(P<0.05);PTEN基因改变与病理分期、发生部位、年龄、性别、乳酸脱氢酶(LDH)水平均无相关性(P>0.05)。结论 PTCL-NOS存在的抑癌基因PTEN杂合性缺失,在PTCL-NOS发生、发展中可能起重要作用,是评估该肿瘤预后的重要指标。     

小细胞性非特指外周T细胞淋巴瘤病理形态和免疫表型分析

目的 探讨小细胞性非特指外周T细胞淋巴瘤(PTCL,NOS)的临床病理与免疫表型及其病理诊断和鉴别诊断.方法 对5例小细胞性PTCL,NOS进行临床病理回顾性研究和随访,免疫表型检测(SP和EnVision法),以及EBER原位杂交和T细胞受体(TCR)基因重排分析.结果 5例均为男性,平均年龄52.6岁.中位病程1个月.5例中3例为临床Ⅳ期,2例为临床Ⅲ期.4例有全身浅表淋巴结及脾脏肿大,1例有肝肿大.2例有浆膜腔积液.行骨髓检查的4例中,3例有肿瘤累及.1例有外周血自细胞总数和淋巴细胞分类计数升高.主要病理改变为淋巴结结构的破坏和单一形态的小淋巴细胞弥漫性浸润,4例可见少数大的异形细胞散在分布,2例见小血管增生现象.5例之肿瘤细胞均表达两种以上T细胞分化抗原和CD43,表达CD99(3/4),均不表达CD20、末端脱氧核苷酸转移酶、CD56和粒酶B.Ki-67指数为5%-15%.4例行TCR基因重排分析,均存在TCRy基因克隆性重排,1例检出TCRβ基因克隆性重排.EBER原位杂交检测均为阴性.获得3例随访资料,且患者均死亡,平均生存时间21.7个月.结论 小细胞性PTCL,NOS少见,呈高临床分期,预后差,组织形态表现为惰性淋巴瘤.     

肠道T细胞淋巴瘤中的EB病毒感染和T细胞内抗原1的表达

目的 探讨ＥＢ病毒感染在肠道Ｔ细胞淋巴瘤发病中的意义。方法 用ＥＢＥＲ１／２原位杂交及三步ＡＢＣ法免疫组织化学染色技术,观察２４例肠道Ｔ淋巴瘤患者中ＥＢ病毒感染及Ｑ细胞内抗原（ＴＩＡ－１）抗原表达情况,选用的抗体有ＴＬＡ－１,ＬＭＰ－１,ＣＤ３,ＣＤ２０,ＣＤ３０和ＣＤ４５ＲＯ等。     

细胞毒性T细胞淋巴瘤134例临床病理学特点及预后

目的:探讨细胞毒性T细胞淋巴瘤（cytotoxic T-cell lymphoma,CTL）临床病理学特点及预后。方法:回顾性收集2008至2020年首都医科大学附属北京友谊医院、解放军联勤保障部队第九八九医院（原第一五二中心医院）和河北医科大学第四医院共计134例CTL患者的临床病理资料,检测肿瘤细胞的免疫表型、EB...     

不同亚型T细胞淋巴瘤与EB病毒的关系

目的 :探讨不同组织学类型T细胞淋巴瘤 (TCL)与EBV感染的关系。方法 :对 83例 (6种类型 )TCL进行研究 ,包括低度恶性 30例 (其中小多形 2 1例、小淋巴细胞性 9例 ) ,高度恶性 5 3例 (其中大 /中多形 31例、淋巴母细胞性 9例、间变性大细胞性 7例、透明细胞性 6例 )。采用PCR检测EBV特征性的DNA序列 (EBV DNA)和ISH法检测EBV编码的RNA(EBER 1/2 )。结果 :每种类型TCL均有EBV的阳性表达 ,低度恶性组与高度恶性组之间EBER 1/ 2表达率差别有显著性 (P <0 0 5 )。结论 :各种类型TCL的发生发展均与EBV感染有关 ,但高度恶性组EBER 1/ 2的表达比低度恶性组更显著。     

皮下脂膜炎样T细胞淋巴瘤的细胞毒颗粒相关蛋白TIA—1的表达及 …

目的 认识皮下脂膜炎样Ｔ细胞淋巴瘤（ＳＰＴＣＬ）的临床和病理特点。研究细胞毒颗粒相关蛋白ＴＬＡ－１在ＳＰＴＣＬ中的表达及与Ｅｐｓｔｅｉｎ－Ｂａｒｒ（ＥＢ）病毒感染的关系。方法 和ＴＩＡ－１、ＣＤ４５ＲＯ、ＣＤ３、ＣＤ２０和ＣＤ６８等抗体作免疫组织化学ＡＢＣ法染色应用ＥＢＥＲ１／２原位杂交检测ＥＢ病的潜伏感染。结果 １７例ＳＰＴＣＬ男女之比１：１．１．中位发病年龄２４岁,主要表现为下肢、躯干无症状性     

EB病毒潜伏感染在不同位T细胞淋巴瘤中的表达

探讨不同部位Ｔ细胞淋巴瘤与ＥＢ病毒感染的关系。方法对１００例不同部位的ＴＭＬ，采用原位杂交法检测肿瘤细胞ＥＢＶ编码的ＲＮＡ。结果（１）１００例中ＥＢＥＲ１／２检出率４８％，结内ＴＬ检出率３０％，结外ＴＭＬ检出率６０％，结内，结外ＥＢＶ检率差异有非常显著意义。     

韦氏环伴滤泡辅助T细胞表型并异常表达CD20的外周T细胞淋巴瘤1例并文献复习

目的 探讨韦氏环伴滤泡辅助T细胞表型的外周T细胞淋巴瘤(Waldeyer’s ring peripheral T-cell lymphoma with T-follicular helper phenotype, wPTCL-TFH)临床病理学、遗传学特征及预后。方法 收集1例wPTCL-TFH临床资料,对其病变组织行HE、免疫组化染色、EBER原位杂交及TCR基因重排检测,应用一代测序法检测IDH2 Exon4基因突变情况,并复习相关文献。结果 患者女性,61岁,临床表现为咽痛、消瘦,无其他发热、盗汗等B症状。组织学表现为扁桃体实质内小-中等大淋巴细胞弥漫浸润。免疫表型：肿瘤细胞表达全T细胞标志物及滤泡辅助T细胞标志物：CD10、BCL6和PD-1,弥漫表达CD20,部分表达CD30(约10%);TCR基因单克隆性重排,IDH2基因未见突变。采用R-CHOP方案化疗6个周期,随访7个月,患者无瘤生存。结论 wPTCL-TFH异常表达CD20者临床罕见,化疗中加入利妥昔单抗可能对CD20异常表达wPTCL-TFH有效,仍需更多的病例进一步证实。     

Comparative analysis of NK/T-cell lymphoma and peripheral T-cell lymphoma in Korea: Clinicopathological correlations and analysis of EBV strain type and 30-bp deletion variant LMP1

Natural killer/T-cell lymphoma (NKTL) and peripheral T-cell lymphomas (PTCL) are prevalent in the Asian population and exhibit a high association with the Epstein-Barr virus (EBV). Moreover, differentiation of these two groups is often difficult and problematic. We investigated 35 cases of NKTL (22 nasal cases and 13 extranasal cases) and 30 cases of PTCL in terms of their clinical features, immunohistology, EBV positivity, EBV strain-type polymorphism and latent membrane protein 1 (LMP1) deletion variant distribution. Eighteen cases (82%) of nasal NKTL and seven (54%) of extranasal NKTL showed EBV positivity by EBV in situ hybridization. Fifteen cases (50%) of PTCL revealed EBV positivity. EBV strain type A was predominant in NKTL (18:5), and EBV strain types A and B were distributed evenly in PTCL (6:6). EBV-positive patients had significantly shorter survival than EBV-negative patients (P < 0.05), and EBV positivity correlated with advanced clinical stage (P < 0.05). Patients harboring type A EBV showed slightly poorer prognoses than those having type B, though it was not obviously statistically different (P = 0.07). The LMP1 deletion variant was prevalent in both NKTL (three wild-type LMP1, 15 deletion variants) and PTCL (three wild-type LMP1, eight deletion variants, two coexistent forms) patients, but did not have prognostic impact. Our results indicate that EBV acts as a negative prognostic factor in NKTL and PTCL, and that the intrinsic properties of a specific viral strain might influence the clinical behavior of these diseases.     

Primary cutaneous peripheral T-cell lymphoma with a late relapse solely in the ileum mimicking monomorphic epitheliotropic intestinal T-cell lymphoma

Background

Primary cutaneous peripheral T-cell lymphomas (PC-PTCLs) are classified into mycosis fungoides (MF) and other rare specific types; and those do not fit into any specific entities are designated as PTCL, not otherwise specified (NOS), an aggressive neoplasm. Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is an aggressive primary intestinal T-cell lymphoma with enteropathy in the non-neoplastic mucosa. We report a rare case of PC-PTCL-NOS with a late relapse solely in the ileum after complete remission. We discuss the importance of evaluating enteropathy, megakaryocyte-associated tyrosine kinase (MATK) immunostaining, and the implication of clonal relationship of metachronous lymphomas.

CXCL13、CD10和bcl-6在血管免疫母细胞性T细胞淋巴瘤的诊断及鉴别诊断中的作用

目的 探讨CXCL13、CD10、bcl-6等标志物在血管免疫母细胞性T细胞淋巴瘤(AITL)的诊断和鉴别诊断中的作用.方法 对四川大学华西医院病理科1990年1月至2008年1月诊断的115例AITL、30例非特指外周T细胞淋巴瘤(PTCL,NOS)和30例以副皮质区增生为主的反应性增生(RH)进行回顾性分析.按2008版WHO关于淋巴造血组织肿瘤分类进行组织学分型,采用9种抗原标志物的免疫组织化学(SP法)染色及TCR-γ基因重排检测.结果 (1)7.8%(9/115)的AITL、6.7%(2/30)的PTCL,NOS和83.3%(25/30)的RH病例观察到生发中心;98.3%(113/115)的AITL、63.3%(19/30)的FTCL,NOS和76.7%(23/30)的RH病例观察到显著血管增生.(2)CXCL13、CD10、bcl-6在RH病例的表达局限在生发中心,在AITL的表达率分别为96.5%(111/115)、50.4%(58/115)和78.3%(90/115),在PTCL,NOS的表达率分别为26.7%(8/30)、3.3%(1/30)和3.3%(1/30),以上三个标记在两种淋巴瘤的表达率差异均具有统计学意义.115例AITL病例均见到滤泡外不规则分布的CD21阳性的滤泡树突状细胞网(FDC).TCR-γ基因克隆性重排在AITL中检出率为83%(83/100).结论 AITL是一种来源于生发中心辅助性T细胞(TFH)的高度侵袭性肿瘤,CXCL13、CD10、bcl-6是AITL诊断和鉴别诊断有用标志物.     

非特异性类固醇细胞瘤临床病理特点

目的探讨非特异性类固醇细胞瘤的临床、病理学特征,以及诊断、鉴别诊断和治疗方法。方法对1例非特异性类固醇细胞瘤进行HE和免疫组化EliVision法染色,并复习文献。结果肿瘤呈实性,境界清楚,偶尔呈分叶状。其肿瘤细胞的形态大致为两种,一种为具有嗜酸胞质颗粒的细胞,另一种为胞质透亮的细胞。此肿瘤的恶性程度与肿瘤大小、核非典型性程度、核分裂象多少、出血、坏死等有关。肿瘤细胞免疫组化α-inhibin、calretinin、Melan-A阳性表达。非特异性类固醇细胞瘤需要与广泛黄素化的颗粒细胞瘤、卵泡膜细胞瘤、富于脂质的Sertoli细胞瘤和原发性或继发性的黑色素瘤相鉴别。治疗方法主要取决于肿瘤的良恶性。结论非特异性类固醇细胞瘤占类固醇细胞肿瘤的56%,是类固醇细胞肿瘤中最常见的类型,免疫组化有助于诊断。     

ALK-negative anaplastic large-cell lymphoma demonstrates similar poor prognosis to peripheral T-cell lymphoma, unspecified

AIMS: Anaplastic large cell lymphoma (ALCL) is classically considered a clinicopathological entity separate from other nodal mature T-cell lymphomas (TCL). Recently, the anaplastic lymphoma kinase (ALK) protein was shown to identify a subgroup of nodal ALCL with an excellent prognosis, whereas ALK-negative ALCLs are more heterogeneous. The aim of this study was to investigate the clinicopathological parameters in relation to clinical behaviour of ALK-negative ALCL compared with other nodal mature TCL, i.e. peripheral TCL, unspecified (PTCL-NOS) and angioimmunoblastic lymphoma (AILT). METHODS AND RESULTS: Clinicopathological data of ALK-positive (n = 28) and ALK-negative (n = 46) ALCL; PTCL-NOS (n = 47); and AILT (n = 12) were analysed for their prognostic significance. While ALK-positive ALCL shows favourable clinical features and a good prognosis, ALK-negative ALCL, PTCL-NOS and AILT are all associated with high age groups, advanced disease stage, and poor prognosis (<45% 5-year survival). In multivariate analysis of overall survival time, performed in the combined group of ALK-negative nodal mature T-cell lymphomas, only age and the International Prognostic Index (IPI) remained independent prognostic parameters, while lymphoma subtype (ALCL versus PTCL-NOS versus AILT) gave no additional information. CONCLUSIONS: The distinction between ALK-negative ALCL and PTCL-NOS or AILT is of limited clinical relevance as they show comparable poor prognosis. In these lymphoma subtypes, only age and the IPI are of significant prognostic value.     

Nodal cytotoxic molecule (CM)-positive Epstein-Barr virus (EBV)-associated peripheral T cell lymphoma (PTCL): a clinicopathological study of 26 cases

Kato S, Takahashi E, Asano N, Tanaka T, Megahed N, Kinoshita T & Nakamura S
(2012) Histopathology  61, 186–199 Nodal cytotoxic molecule (CM)‐positive Epstein–Barr virus (EBV)‐associated peripheral T cell lymphoma (PTCL): a clinicopathological study of 26 cases Aims: The clinicopathological distinctiveness of nodal cytotoxic molecule (CM)‐positive Epstein–Barr virus (EBV)‐associated peripheral T cell lymphoma (PTCL) remains to be clarified. We investigated 26 patients with this lymphoma compared to nodal CM⁺ EBV^? PTCL (n = 39) and extranasal natural killer/T cell lymphoma of nasal type (ENKTL, n = 44). Methods and results: Nodal CM⁺ EBV⁺ PTCL patients were more likely to have B symptoms (P = 0.019) and hepatic involvement (P = 0.026) than nodal CM⁺ EBV^? PTCL patients. The former also had more Stage III/IV disease (P = 0.025) but much less cutaneous involvement (P < 0.001) than ENKTL patients at diagnosis. This nodal EBV⁺ lymphoma possessed a distinctive immunophenotype of high CD8⁺, CD56^? pattern with an aggressive clinical course (median, 6.6 months). Thrombocytopenia was present in 11 (50%) patients and found to be the strongest prognostic indicator (P = 0.001) in this nodal EBV⁺ group. For all nodal CM⁺ PTCL cases CD5 negativity, but not EBV positivity, was the significant adverse prognostic factor (P < 0.002) in a multivariate analysis, independent of prognostic index for PTCL (PIT) or International Prognostic Index (IPI) scores. Conclusions: Nodal CM⁺ EBV⁺ PTCL constitutes a unique group of lymphomas distinct from ENKTL. The data provide support for our assertion that nodal CM⁺ PTCL should be distinguished in the 2008 WHO category of PTCL, not otherwise specified.