血管内NK/T细胞淋巴瘤(intravascular NK/T cell lymphoma,IVNKTL)罕见,其特征性表现为肿瘤性淋巴细胞选择性生长于血管腔内。不同地域患者临床表现多样且缺乏特异性,因此临床诊断困难。IVNKTL具有病程进展快、伴EBV感染、好发于亚洲,且以中国人多发等特点。在肿瘤未累及到多器官前进行早期诊治可有效改善IVNKTL患者的预后。
Intravascular NK/T cell lymphoma(IVNKTL) is a rare type of malignant lymphoma characterized by the selective growth of neoplastic lymphocytes within the lumina of blood vessels. The clinical manifestations are diverse and non-specific in different geographical areas, making diagnosis difficult. IVNKTL is a highly aggressive and EBVassociated lymphoma that is prone to develop in Asian patients, particularly in Chinese patients. Early diagnosis and treatment before multi-organ involvement could effectively improve the prognosis of IVNKTL patients.