CAJ | 학술논문

目的：提高对皮肤血管内NK/T细胞淋巴瘤（CIVNKTC）的认识。方法回顾分析5例CIVNKTC的临床资料、组织病理学、治疗及预后，并进行讨论。结果5例患者中男1例，女4例，发病年龄38~83岁（平均56．2岁），均以多发皮肤斑块或结节为初发表现。组织病理显示：真皮及皮下组织内可见血管扩张，扩张的血管内可见异形淋巴样细胞，细胞核大，可见1~2个小核仁。肿瘤细胞表达CD3ε，细胞毒蛋白（T细胞限制性细胞内抗原-1，粒酶B和穿孔素）和EB病毒编码的小RNA，不表达细胞角蛋白CK，CD20，CD79A，CD4，CD8。2例患者表达CD56。5例患者中2例进行化疗，3例未治疗。经过24个月的随访，4例死亡，仅有1例带病生存。结论CIVNKTC是一种罕见的结外霍奇金淋巴瘤，具有独特的组织学和免疫表型特点，病程凶险，预后极差。
목적：제고대피부혈관내NK/T세포림파류（CIVNKTC）적인식。방법회고분석5례CIVNKTC적림상자료、조직병이학、치료급예후，병진행토론。결과5례환자중남1례，녀4례，발병년령38~83세（평균56．2세），균이다발피부반괴혹결절위초발표현。조직병리현시：진피급피하조직내가견혈관확장，확장적혈관내가견이형림파양세포，세포핵대，가견1~2개소핵인。종류세포표체CD3ε，세포독단백（T세포한제성세포내항원-1，립매B화천공소）화EB병독편마적소RNA，불표체세포각단백CK，CD20，CD79A，CD4，CD8。2례환자표체CD56。5례환자중2례진행화료，3례미치료。경과24개월적수방，4례사망，부유1례대병생존。결론CIVNKTC시일충한견적결외곽기금림파류，구유독특적조직학화면역표형특점，병정흉험，예후겁차。
Objective To improve the understanding of cutaneous intravascular natural killer/T-cell lymphoma (CIVNKTC). Methods Clinical data on five cases of CIVNKTC were collected. The histopathological feature, treatment and prognosis of CIVNKTC were retrospectively analyzed and discussed. Results Of the 5 patients, 1 was male and 4 were female. The age of onset ranged from 38 to 83 years (average, 56.2 years). All the patients presented with multiple plaques and nodules as the primary symptoms. Histopathological examination revealed vasodilatation in the dermis and subcutaneous tissue, as well as atypical lymphoid cells with large hyperchromatic nuclei containing 1-2 small nucleoli in dilated veins. Immunohistochemical studies of tumor cells showed positive staining for CD3ε, cytotoxic proteins (including T cell-restricted intracellular antigen-1, granzyme B and perforin)and Epstein-Barr virus(EBV)-encoded microRNA, but negative staining for cytokeratin, CD20, CD79a, CD4 and CD8. Furthermore, the tumor cells stained positive for CD56 in two patients. Among the 5 patients, only 2 received chemotherapy and the remaining received no treatment. During a 24-month follow-up, 4 patients died, and only 1 survived with the tumor. Conclusion CIVNKTC is a rare extranodal Hodgkin′s lymphoma with distinct histologic manifestations and immunophenotypes, rapid and aggressive clinical course, and poor prognosis.