Department of Histopathology, Royal Marsden Hospital, London, UK.
Histopathology. 2014 Jan;64(2):171-99. doi: 10.1111/his.12251. Epub 2013 Oct 16.
Mature T-cell and T/NK-cell neoplasms are both uncommon and heterogeneous, among the broad category of non-Hodgkin lymphomas. Owing to the lack of specific genetic alterations in the vast majority, most currently defined entities show overlapping morphological and immunophenotypic features, and therefore pose a challenge to the diagnostic pathologist. In the light of recent immunophenotypic, cytogenetic and molecular genetics advances in the field of T-cell and T/NK-cell lymphomas, the focus of the lymphoma workshop of the European Association for Haematopathology/Society for Hematopathology meeting in Lisbon, Portugal, in October 2012 was to refine existing diagnostic criteria and clarify the borders between overlapping entities. The panel reviewed over 200 submitted cases, which were grouped into five categories: (i) angioimmunoblastic T-cell lymphoma and T-follicular-helper-cell-associated lymphomas; (ii) CD30-positive T-cell lymphomas/lymphoproliferative diseases; (iii) extranodal T-cell and NK-cell neoplasms; (iv) EBV-associated T-cell/NK-cell lymphomas/lymphoproliferative diseases; and (v) peripheral T-cell lymphoma, not otherwise specified, post-transplant lymphoproliferative disorders, and mimics. This report summarizes the discussions and conclusions of the workshop, which question current diagnostic criteria and provide recommendations for refining existing classifications.
成熟 T 细胞和 T/NK 细胞肿瘤均不常见且具有异质性,属于非霍奇金淋巴瘤的广泛类别。由于绝大多数肿瘤缺乏特异性的遗传改变,大多数目前定义的实体具有重叠的形态学和免疫表型特征,因此对诊断病理学家构成了挑战。鉴于近年来 T 细胞和 T/NK 细胞淋巴瘤领域的免疫表型、细胞遗传学和分子遗传学的进展,2012 年 10 月在葡萄牙里斯本举行的欧洲血液病理学协会/血液病理学学会淋巴瘤研讨会的淋巴瘤工作组的重点是完善现有的诊断标准并阐明重叠实体之间的界限。专家组审查了超过 200 例提交的病例,这些病例被分为五类:(i)血管免疫母细胞性 T 细胞淋巴瘤和 T 滤泡辅助细胞相关性淋巴瘤;(ii)CD30 阳性 T 细胞淋巴瘤/淋巴增生性疾病;(iii)结外 T 细胞和 NK 细胞肿瘤;(iv)EBV 相关 T 细胞/NK 细胞淋巴瘤/淋巴增生性疾病;以及(v)外周 T 细胞淋巴瘤,非特指型,移植后淋巴组织增生性疾病和类似物。本报告总结了研讨会的讨论和结论,这些讨论和结论对当前的诊断标准提出了质疑,并为完善现有分类提出了建议。