摘要
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。
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 histopathologieal 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. Histopathologieal examination revealed vasodilatation in the dermis and subcutaneous tissue, as well as atypical lymphoid cells with large hyperehromatie nuclei containing 1 - 2 small nueleoli in dilated veins. Immunohistoehemical studies of tumor cells showed positive staining for CD3e, cytotoxic proteins （including T cell-restricted intracellular antigen-l, granzyme B and perforin） and Epstein-Barr virus （EBV）-eneoded 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 histologie manifestations and immunophenotypes, rapid and aggressive clinical course, and poor prognosis.
出处
《中华皮肤科杂志》
CAS
CSCD
北大核心
2015年第9期603-605,共3页
Chinese Journal of Dermatology
关键词
淋巴瘤
结外NK-T细胞
皮肤表现
病理过程
免疫组织化学
血管内淋巴瘤
Lymphoma, extranodal NK-T-eell
Skin manifestations
Pathological processes
Immunohistochemistry
Intravaseular lymphoma
