结外NK/T细胞淋巴瘤治疗新进展

Advances in the treatment of extranodal NK/T-cell lymphoma

结外NK/T细胞淋巴瘤[extranodal natural killer(NK)/T-cell lymphoma,ENKTL]具有独特的地域分布,病理特征为显著血管破坏和组织坏死。典型的NK细胞亚型一般表达CD2+,CD3(ε)+,CD56+,EBV+。MRI检查可以帮助确定局部病灶侵犯情况,PET/CT检查可以帮助了解病变的受累情况。目前国际上普遍使用国际预后指数(IPI)作为预后因子,同时行血EBV-DNA测定。对于局限于鼻腔的病变,即使在化疗有效的基础上,放疗仍具有非常重要的作用。超过一半的患者可以获得持续缓解,鼻型和播散型NK/T细胞淋巴瘤通常预后较差,目前以系统性全身化疗的治疗方案为主流。含蒽环类药物的方案疗效并不令人满意,含左旋门冬酰胺酶及其他新药的治疗方案的研究正在进行中。预后不良的患者应考虑早期行自体造血干细胞移植。异基因移植治疗本病有效,但移植相关死亡率也较高。

Extranodal natural killer（NK）/T-cell lymphoma（ENKTL）has a unique geographic distribution.Its pathology is marked characterized by angioinvasion,prominent necrosis,expression of cytoplasmic CD3 epsilon（ε）,CD56 and with Ebstein-Barr virus infection.Magnetic resonance imaging can help to clearly define the local involvement.Positron emission tomography can help to demonstrate the system spread.Prognostic variable IPI should be documented.Plasma EBV-DNA should be quantified at the same time.For local disease,radiotherapy is important,although chemotherapy is added.Sustainable remission is observed in over half of the patients.For extranasal type and disseminated disease,systemic chemotherapy becomes the mainstay.Doxorubicin containing regimens are not entirely satisfactory and L-asparaginase containing regimens are being investigated.Patients with poor prognostic features may be considered for an early autologous haematopoietic stem cell transplant.New therapeutic method is urgently needed.