Warnnissorn Naree, Kanitsap Nonglak, Kulkantrakorn Kongkiat, Assanasen Thamathorn
Department of Pathology, Faculty of Medicine, Thammasat University, Klongloung, Pathumthani 12120, Thailand.
J Med Assoc Thai. 2007 May;90(5):982-7.
Natural killer cell malignancy is a rare and aggressive lymphoid neoplasm encompassing extra-nodal NK/T-cell lymphoma, nasal-type (ENKLN) and aggressive NK-cell lymphoma/leukemia (ANKL). A case of cutaneous ENKLN and a case of ANKL in Thai patients are reported Both patients developed hemophagocytic syndrome and shortly succumbed to death. The cells in cutaneous ENKLN are small to medium in size with minimal cytoplasm, round nuclei, irregular nuclear membrane, andfine chromatin with inconspicuous nucleoli. While that of ANKL are medium to large-sized mononuclear cells with moderate cytoplasm. Their nuclei are elongated to embryo-like with irregularly thickened nuclear membrane, fine chromatin, and small to occasional prominent nucleolus. Ancillary techniques studied on paraffin embedded tissues of both cases demonstrated that the neoplastic cells exhibit cytoplasmic CD3+, CD56+ and cytotoxic granules + by immunohistochemistry, absence of T cell receptor gene rearrangement by PCR, and presence of Epstein-Barr virus mRNA (EBER) transcripts by in situ hybridization. The authors reviewed the literature on natural killer cell neoplasm and compared the clinical characteristics, natural history, and association of Epstein-Barr virus infection with hemophagocytic syndrome.
自然杀伤细胞恶性肿瘤是一种罕见且侵袭性的淋巴样肿瘤，包括结外NK/T细胞淋巴瘤，鼻型（ENKLN）和侵袭性NK细胞淋巴瘤/白血病（ANKL）。本文报道了两例泰国患者，一例为皮肤ENKLN，另一例为ANKL。两名患者均发生噬血细胞综合征并很快死亡。皮肤ENKLN中的细胞大小为小到中等，细胞质极少，细胞核圆形，核膜不规则，染色质细，核仁不明显。而ANKL的细胞为中等至大的单核细胞，细胞质中等。它们的细胞核拉长呈胚胎样，核膜不规则增厚，染色质细，核仁小至偶尔明显。对两例病例石蜡包埋组织进行的辅助技术研究表明，肿瘤细胞通过免疫组织化学显示细胞质CD3 +、CD56 +和细胞毒性颗粒+，通过PCR检测无T细胞受体基因重排，通过原位杂交检测有爱泼斯坦-巴尔病毒mRNA（EBER）转录本。作者回顾了关于自然杀伤细胞肿瘤的文献，并比较了临床特征、自然病史以及爱泼斯坦-巴尔病毒感染与噬血细胞综合征的关联。
