Peripheral T cell lymphoma, Not otherwise specified

  • 文章类型: Journal Article
    本研究旨在确定外周T细胞淋巴瘤的临床病理预测因素。未指定(PTCL,NOS),结性T滤泡辅助细胞淋巴瘤,血管免疫母细胞型(nTFH,AI型)。在这个单一的中心,回顾性研究,59名诊断为PTCL的患者的医疗记录,NOS,或nTFH,回顾了2007年3月至2022年9月的AI类型。临床病理变量,包括免疫组织化学(IHC)亚组,分析了TBX21与GATA3亚组的区别.总的来说,TBX21组28例(75.7%)患者行PTCL,NOS.GATA3组有9例(24.3%)患者。在单变量分析中,淋巴瘤亚型,年龄,和表现状态与无进展生存期(PFS)相关,总生存率(OS)。在多变量分析中,淋巴瘤亚型,和表现状态与PFS和OS相关(分别为P=0.012,P<0.001,P=0.006和P<0.001)。在单变量分析中,GATA3亚组的预后往往较差;然而,当调整淋巴瘤亚型和表现状态时,其在多变量中变得更加不显著(分别为P=0.065,P=0.180,P=0.972和P=0.265).双阳性组表现出不同的预后,即PFS较好,OS较差。PD-1和PD-L1与EBV原位杂交相关(P=0.027,P=0.005),PD-1与CD30表达相关(P=0.043)。这项研究证明了IHC分类预测PTCL预后的潜力,NOS,以及nTFHAI型,虽然进一步验证是必要的。靶向CD30、PD-1和PD-L1的治疗对于淋巴瘤治疗似乎是有希望的。
    This study aimed to determine the clinicopathological predictive factors of peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS), and nodal T-follicular helper cell lymphoma, angioimmunoblastic-type (nTFH, AI-type). In this single-centered, retrospective study, medical records of 59 patients who were diagnosed with PTCL, NOS, or nTFH, AI-type from March 2007 to September 2022 were reviewed. The clinicopathological variables, including immunohistochemistry(IHC) subgroups, distinguishing TBX21 from the GATA3 subgroups were analyzed. Overall, 28 patients (75.7%) in the TBX21 group were PTCL, NOS. There were 9 (24.3%) patients in the GATA3 group. In univariable analyses, lymphoma subtype, age, and performance status were associated with progression-free survival (PFS), and overall survival (OS). In multivariable analyses, lymphoma subtype, and performance status were related to PFS and OS (P = 0.012, P < 0.001, P = 0.006, and P < 0.001, respectively). The GATA3 subgroup tended to have a worse prognosis in univariable analyses; however, it became more insignificant in multivariable when lymphoma subtype and performance status were adjusted (P = 0.065, P = 0.180, P = 0.972, and P = 0.265, respectively). The double-positive group showed variable prognoses of better PFS and worse OS. PD-1 and PD-L1 were associated with the EBV in situ hybridization (P = 0.027, and P = 0.005), and PD-1 was associated with CD30 expression (P = 0.043). This study demonstrated the potential of IHC classification to predict prognosis for PTCL, NOS, as well as nTFH AI-type, although further validation is necessary. Treatments targeting CD30, PD-1, and PD-L1 appear promising for lymphoma treatment.
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  • 文章类型: Journal Article
    外周T细胞淋巴瘤,未指定(PTCL,NOS),是一种异质性和侵袭性的非霍奇金淋巴瘤,预后暗淡。本研究旨在评估EFS24作为替代临床终点的价值,并确定PTCL的预后相关因素。NOS.诊断为PTCL的患者,回顾性收集NOS,并由两名血液病理学家审查了载玻片。EFS定义为从诊断到初始治疗后疾病进展发生的时间,再治疗,或死亡。随后的总生存期(OS)从EFS24或进展时间定义,如果发生在24个月内,最后一次随访或死亡。选择随访完整的患者97例。约66例患者(68.04%)未能达到ES24,中位OS为12.17个月,5年OS率为15.17%。而达到EFS24的患者的中位OS为60.57个月,5年OS率为68.77%。多因素Cox分析表明,骨髓受累和β2微球蛋白(β2-MG)升高与预后不良有关。B症状,结外受累不止一个部位,高Ki67指数是预测EFS24失效的重要因素。EFS24可以帮助对PTCL的后续结果进行分层,NOS.达到EFS24的患者预后良好,虽然它没有达到普通人群的水平。另一方面,未达到EFS24的患者预后极差.因此,EFS24可用于患者风险分层,病人咨询,和研究设计。
    Peripheral T cell lymphoma, not otherwise specified (PTCL, NOS), is a heterogeneous and aggressive type of non-Hodgkin\'s lymphoma with a bleak prognosis. This study was designed to assess the value of EFS24 as an alternative clinical endpoint and identify prognosis-related factors in PTCL, NOS. Patients diagnosed with PTCL, NOS were retrospectively collected and slides were reviewed by two hematopathologists. EFS was defined as the time from diagnosis to the occurrence of disease progression after initial treatment, retreatment, or death. Subsequent overall survival (OS) was defined from EFS24 or time of progression, if it occurred within 24 months, to the last follow-up or death. 97 cases with complete follow-up were selected. Approximately 66 patients (68.04%) failed to achieve ES24, with the median OS of 12.17 months, and 5-year OS rate of 15.17%. While patients who reached EFS24 had a median OS of 60.57 months and a 5-year OS rate of 68.77%. Multivariate Cox analysis indicated that bone marrow involvement and elevated β2 Microglobulin (β2-MG) were associated with a poor prognosis. B symptoms, extranodal involvement more than one site, and a high Ki67 index were significant factors in predicting the failure of EFS24. EFS24 can help stratify the subsequent outcomes of PTCL, NOS. Patients who achieve EFS24 have a favorable prognosis, although it does not reach that of the general population. On the other hand, patients who do not achieve EFS24 have an extremely poor prognosis. Therefore, EFS24 can be used for patient risk stratification, patient counseling, and study design.
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  • 文章类型: Journal Article
    Peripheral T cell lymphoma, not otherwise specified (PTCL-NOS), is a heterogeneous disease with respect to clinicopathological features. Cell adhesion molecule 1 (CADM1) has been reported to be ectopically expressed in adult T cell leukaemia/lymphoma (ATLL). However, the frequency of CADM1 expression remains unknown in peripheral T cell lymphomas. In the current study, CADM1 expression was analysed in 88 PTCL-NOS patients. CADM1 was expressed in 14 of 88 (15.9%) PTCL-NOS cases, and its expression was associated with C-C chemokine receptor type 4 (CCR4) expression and nuclear atypia. CADM1-positive PTCL-NOS cases (10/74) had a significantly poorer prognosis than CADM1-negative cases (64/74) (P = 0.001). Multivariate analysis confirmed that CADM1 expression was an independent prognostic factor in PTCL-NOS. These findings suggest that CADM1 expression is a novel prognostic factor for PTCL-NOS.
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  • 文章类型: Journal Article
    Clinical differences between anaplastic lymphoma kinase (ALK)-negative anaplastic large-cell lymphoma (ALK(-) ALCL) and peripheral T cell lymphoma, not otherwise specified (PTCL-NOS), remain unclear. The aim of this study was to compare the clinical and prognostic features of these two lymphoma types. We retrospectively analyzed 167 patients with ALK(-) ALCL (n = 48) and PTCL-NOS (n = 119). Compared with ALK(-) ALCL patients, PTCL-NOS patients exhibited distinct differences in clinical features with a propensity for more advanced stages, frequent extranodal involvement, and a poor performance status, leading to a higher risk group according to the International Prognostic Index or Prognostic Index for PTCL-NOS. Patients with ALK(-) ALCL were associated with a higher complete response rate (47.9 vs. 31.0 %; P = 0.041) after initial chemotherapy than patients with PTCL-NOS. The prognosis was significantly different between two subtypes, with a 5-year overall survival (OS) rate of 57.9 % for ALK(-) ALCL and 23.9 % for PTCL-NOS (P = 0.002). The subgroup analysis showed significant differences in OS and progression-free survival between the two subtypes in early-stage diseases, but not in advanced-stage diseases. We conclude that patients with ALK(-) ALCL showed favorable clinical features, higher chemosensitivity, and a superior outcome than those with PTCL-NOS.
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