Institute of Pathology, Klinikum rechts der Isar, Technische Universität München, München, Germany.
Am J Surg Pathol. 2010 Sep;34(9):1382-7. doi: 10.1097/PAS.0b013e3181ec577a.
We report a case of primary Epstein Barr virus (EBV) negative peripheral T-cell lymphoma (PTCL) NOS in a 56-year-old female who-after an initially indolent course - simultaneously developed an aggressive, EBV+ cytotoxic large T-cell lymphoma, clonally related to the primary PTCL, and an EBV+, clonal large B-cell lymphoproliferation. The initial, EBV-negative PTCL had shown some features of angioimmunoblastic T-cell lymphoma and had responded well to steroid therapy. Two years later, rapidly fatal, progressive disease with multivisceral involvement developed. Histologically, extensive infiltrates of EBV+, CD8+ large cells were present, in addition to areas of the initial PTCL. Extensive comparative phenotypic and molecular analyses confirmed the presence of an identical CD8+ T-cell clone in the initial EBV-negative PTCL and the EBV+, CD8+ large cell lymphoma at the time of aggressive transformation. These results also justified the retrospective classification of PTCL, NOS for the initial lymphoma. This case shows that secondary EBV infection of an established malignant T-cell clone can occur and may contribute to aggressive transformation of PTCL.
我们报告了一例 56 岁女性原发性 EBV 阴性外周 T 细胞淋巴瘤（PTCL）NOS，其在初始惰性病程后同时发生了侵袭性 EBV+细胞毒性大 T 细胞淋巴瘤，与原发性 PTCL 克隆相关，以及 EBV+克隆性大 B 细胞淋巴增生。初始的 EBV 阴性 PTCL 具有一些血管免疫母细胞性 T 细胞淋巴瘤的特征，对类固醇治疗反应良好。两年后，出现了多器官受累的快速致命、进行性疾病。组织学上，除了原发性 PTCL 区域外，还存在广泛的 EBV+、CD8+大细胞浸润。广泛的比较表型和分子分析证实，在初始 EBV 阴性 PTCL 和侵袭性转化时的 EBV+、CD8+大细胞淋巴瘤中存在相同的 CD8+T 细胞克隆。这些结果也证明了初始淋巴瘤的 PTCL，NOS 的回顾性分类是合理的。该病例表明，已建立的恶性 T 细胞克隆的继发 EBV 感染可能会发生，并可能导致 PTCL 的侵袭性转化。
