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Abstract：
The nasal type of extranodal natural killer (NK)/T-cell lymphoma (ENKL) is an aggressive form of non-Hodgkin\'s lymphoma. Although ENKL is most commonly seen in the midline of the nose, face and Waldeyer\'s ring, it can also occur in the skin, gastrointestinal tract, soft tissues and other parts of the body. Severe ENKL cases are accompanied by hemophagocytosis, with clinical manifestations such as high fever, hepatosplenomegaly, and decreased blood cell count. ENKL at different locations exhibits similar histological features and immunophenotypes, such as a strong affinity for T cell markers CD2 and CD56, cytotoxic molecules, as well as a strong positive for EBER after in situ hybridization. Although indolent ENKL is extremely rare, we hereby present a case study of primary NK/T cell lymphoma in the spinal canal with the initial manifestation of a diffuse growth of small cells, and the survival and recurrence details after 11 years, accompanied by CD30-positive large cell transformation. The patient\'s condition after treatment has improved and is currently in good health.
摘要：
结外自然杀伤（NK）/T细胞淋巴瘤（ENKL）的鼻型是非霍奇金淋巴瘤的侵袭性形式。虽然ENKL最常见于鼻子中线，脸和Waldeyer的戒指，它也可以发生在皮肤上，胃肠道,软组织和身体的其他部位。严重的ENKL病例伴有吞噬作用，临床表现如高烧，肝脾肿大,血细胞计数下降。不同位置的ENKL表现出相似的组织学特征和免疫表型，例如对T细胞标志物CD2和CD56，细胞毒性分子的强亲和力，以及原位杂交后EBER的强阳性。虽然惰性ENKL极为罕见，我们在此提出一个病例研究的原发性NK/T细胞淋巴瘤在椎管内，最初表现为弥漫性生长的小细胞，以及11年后的生存和复发细节，伴有CD30阳性大细胞转化。治疗后患者病情好转，目前身体健康。
