Cutaneous intravascular natural killer/T cell lymphoma with peculiar immunophenotype
Cutaneous intravascular natural killer/T cell lymphoma with peculiar immunophenotype
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DOI：
10.1111/his.13332
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发表时间：
2017-12-01
期刊：
影响因子：
6.4
通讯作者：
Rodriguez-Pinilla, Socorro M.
中科院分区：
文献类型：
--
作者：
Alegria-Landa, Victoria;Manzarbeitia, Felix;Rodriguez-Pinilla, Socorro M.
血管内淋巴瘤（IVL）是一种罕见的实体。大多数病例是结外弥漫性大 B 细胞淋巴瘤的变异型，已发表的病例中只有不到 10% 是 T 细胞起源的。在最新的世界卫生组织 (WHO) 淋巴组织增生性疾病分类中，只有血管内 B 细胞淋巴瘤被认为是一个独特的实体。我们描述了一例皮肤自然杀伤 (NK)/T IVL 病例，具有细胞毒性免疫表型和 Epstein-Barr 病毒 (EBV) 阳性。然而，我们的病例不仅 T 细胞受体 (TCR)-F1 和 TCR-（TCR-沉默）免疫组织化学呈阴性，CD56 也呈阴性，这使其成为第一个被描述的三阴性 NK/T IVL 病例。由于这种 NK/T 细胞谱系血管内淋巴瘤具有特殊的免疫表型特征及其与 EBV 不变的关系，我们敦促人们认识到这种 NK/T 细胞谱系血管内淋巴瘤。它的发生不应被认为是巧合，而是这种血液肿瘤致病背景的一个关键方面。
Intravascular lymphoma (IVL) is a rare entity. Most cases are a variant of extranodal diffuse large B cell lymphoma, and fewer than 10% of the published cases are of T cell origin. Only intravascular B cell lymphoma is recognized as a distinct entity in the most recent World Health Organization (WHO) classification of lymphoproliferative disorders. We describe a case of cutaneous natural killer (NK)/T IVL, with a cytotoxic immunophenotype and Epstein-Barr virus (EBV) positivity. However, our case was immunohistochemically negative not only for T cell receptor (TCR)-F1 and TCR- (TCR-silent), but also for CD56, making it the first triple-negative NK/T IVL case to be described. We urge recognition of this NK/T cell lineage intravascular lymphoma due to its particular immunophenotypical profile and its unvarying relationship with EBV. Its occurrence should not be considered a coincidence, but rather a key aspect of the pathogenic background of this haematological neoplasm.