结外NK/T细胞淋巴瘤治疗进展

Advances of treatment for extranodal natural killer / T- cell lymphoma

结外NK/T细胞淋巴瘤(ENKTL)是非霍奇金淋巴瘤中较为罕见的亚型,东亚地区比西方国家多见。主要发生在鼻腔或鼻窦区,皮肤受累少见。基于ENKTL的发病机理,几乎所有患者的淋巴瘤细胞中都存在EB病毒。EBV-DNA拷贝数与肿瘤负荷密切相关,并能预测ENKTL预后。由于P-糖蛋白的表达,使细胞产生耐药性,ENKTL预后较差。近年来,新的治疗方法的研究延长了ENKTL患者的生存期。对于局限期ENKTL放疗联合化疗已取得良好的疗效。对于晚期复发难治ENKTL以L-门冬酰胺酶为基础的化疗方案显著提高治疗有效率。有文献报道,造血干细胞移植在ENKTL的治疗中取得了重要地位,而靶向药物的出现为晚期ENKTL提供了新选择。本文综述了ENKTL的治疗进展。

Extranodal natural killer/T- cell lymphoma（ ENKTL） is one of the uncommon subtypes of malignant lymphoma,and is more prevalent in East Asia than in Western countries. It predominantly occurs in the nasal or paranasal areas and less frequently in the skin. Epstein- Barr virus（ EBV） is present in lymphoma cells of almost all patients,accounting for the pathogenesis of ENKTL. The EBV- DNA copy numbers are associated with tumor burden,and can predict the prognosis of ENKTL. Previously,its prognosis was poor due to the expression of P- glycoprotein,which actively exports several antica ncer agents outside the lymphoma cells. However,in recent years,novel therapeutic method improved the survival of ENKTL patients. A good effect of ENKTL limited stages combined with radiotheraty and chemotherapy has been achieved. Chemotherapy for advanced refractory recurrent ENKTL with L- asparaginase- based significantly improve the treatment efficiency. It is reported that hematopoietic stem cell transplantation（ HSCT） in the treatment of ENKTL has achieved an important position. Targeting therapy is also an optimal choice.This paper reviews the advance of the treatment of ENKTL.