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目的提高对 NK 样 T 细胞淋巴瘤/白血病的认识。方法报道1例 NK 样 T 细胞淋巴瘤/白血病患者,并复习文献,总结该病临床及实验室检查特点。结果患者以发热、皮肤疱疹起病,肿瘤细胞主要分布于皮下小血管周围和血管腔内,免疫表型呈 CD3(+),CD8(+),CD4(-),CD5(-),CD10(-),CD19(-),CD57(-),CD56(+),穿孔素(+)和颗粒酶 B(+),T 细胞受体γ重排阳性。经糖皮质激素治疗一度好转,但很快复发,进展为 NK 样 T 细胞白血病,病程1年,死于多器官功能衰竭。结论 NK 样 T 细胞淋巴瘤/白血病非常少见,具有独特的临床、组织病理学和免疫表型特征,对现有治疗不敏感,预后恶劣。
Objective To improve the understanding of NK-like T-cell lymphoma / leukemia. Methods One patient with NK-like T-cell lymphoma / leukemia was reported. The literature was reviewed and the clinical and laboratory features of the disease were summarized. Results The patients developed fever and skin herpes. Tumor cells were mainly distributed in the subcutaneous small vessels and blood vessels. The immunophenotypes were CD3 (+), CD8 (+), CD4 (-), CD5 (-), CD10 -), CD19 (-), CD57 (-), CD56 (+), perforin (+) and granzyme B (+). Glucocorticoid treatment was once improved, but soon relapsed, progress to NK-like T-cell leukemia, duration of 1 year, died of multiple organ failure. Conclusions NK-like T-cell lymphoma / leukemia is rare and has unique clinical, histopathological and immunophenotypic characteristics that are insensitive to current treatment and have a poor prognosis.