2022年在意大利佛罗伦萨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中，讨论了细胞毒性外周T细胞淋巴瘤和EBV阳性T/NK细胞淋巴增生性疾病。本次会议重点讨论了以下内容：(i) EBV阳性T和NK细胞淋巴瘤的原发性淋巴结型（原发性EBV-TNKL）；(ii) 儿童和成人的EBV阳性T/NK细胞淋巴增生性疾病（LPD）在异位部位发生；(iii) 细胞毒性外周T细胞淋巴瘤，未指明的类型（cPTCL-NOS），EBV阴性；(iv) 杂项病例。原发性EBV-TNKL是一种新近认识的少见、侵袭性疾病，常伴有潜在的免疫缺陷/免疫调节失衡。所有病例均表现为淋巴结肿大，但其中一些还涉及扁桃体/瓦尔代尔环和异位部位。大多数肿瘤为T细胞系，最常见的突变涉及表观遗传调控的基因，如TET2和DNMT3A以及JAK-STAT基因。讨论了涉及异位部位的一系列EBV阳性T/NK细胞淋巴增生性疾病，并强调了当这些异位EBV阳性T/NK细胞淋巴增生性疾病表现出优势的淋巴结病变时，对于原发性EBV-TNKL的诊断挑战，无论是在初次发病时还是在慢性EBV活动性疾病的病程进展中。大多数cPTCL-NOS表现为TBX21表型。一些病例伴有免疫抑制或免疫调节失衡的背景。有趣的是，在研讨会病例中观察到了cPTCL-NOS与TFH淋巴瘤/LPD（EBV阳性和阴性）的意外关联，这与已发表的文献类似。与研讨会的遗传学情况类似，cPTCL-NOS的遗传景观显示常见的表观遗传调节器的突变，包括TET2和DNMT3A，提示克隆性造血中的作用与疾病发病机制有关。©2023. 作者。

Cytotoxic peripheral T-cell lymphomas and EBV-positive T/NK-cell lymphoproliferative diseases were discussed at the 2022 European Association for Haematopathology/Society for Hematopathology lymphoma workshop held in Florence, Italy. This session focused on (i) primary nodal EBV-positive T and NK-cell lymphomas (primary nodal-EBV-TNKL), (ii) extranodal EBV-positive T/NK lymphoproliferative diseases (LPD) in children and adults, (iii) cytotoxic peripheral T-cell lymphomas, NOS (cPTCL-NOS), EBV-negative, and (iv) miscellaneous cases. Primary nodal-EBV-TNKL is a newly recognized entity which is rare, aggressive, and associated with underlying immune deficiency/immune dysregulation. All cases presented with lymphadenopathy but some demonstrated involvement of tonsil/Waldeyer's ring and extranodal sites. The majority of tumors are of T-cell lineage, and the most frequent mutations involve the epigenetic modifier genes, such as TET2 and DNMT3A, and JAK-STAT genes. A spectrum of EBV-positive T/NK LPD involving extranodal sites were discussed and highlight the diagnostic challenge with primary nodal-EBV-TNKL when these extranodal EBV-positive T/NK LPD cases demonstrate predominant nodal disease either at presentation or during disease progression from chronic active EBV disease. The majority of cPTCL-NOS demonstrated the TBX21 phenotype. Some cases had a background of immunosuppression or immune dysregulation. Interestingly, an unexpected association of cPTCL-NOS, EBV-positive and negative, with TFH lymphomas/LPDs was observed in the workshop cases. Similar to a published literature, the genetic landscape of cPTCL-NOS from the workshop showed frequent mutations in epigenetic modifiers, including TET2 and DNMT3A, suggesting a role of clonal hematopoiesis in the disease pathogenesis.© 2023. The Author(s).