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style=\"font-size: 15px;\"> 结外NK\u002FT细胞淋巴瘤（extranodal NK\u002FT-cell lymphoma, ENKTCL）是一种少见的、高侵袭性的非霍奇金淋巴瘤，它起源于NK细胞和T淋巴细胞，恶性程度高、临床进展快，目前针对该疾病还没有高效、特异的治疗手段，预后差。\u003C\u002Fspan>\u003C\u002Fp>\u003Cp>\u003Cbr\u002F>\u003C\u002Fp>\u003Cp>\u003Cspan style=\"font-size: 15px;\">结外NK\u002FT细胞淋巴瘤的发病具有明显的地域和种族差异性，亚洲人群相对高发；患者以中青年常见，男性多于女性。\u003C\u002Fspan>\u003C\u002Fp>","⚕️","2026-08-03",{"key":90,"title":91,"html":92,"icon":93,"updatedAt":88},"cause","病因","\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">结外NK\u002FT细胞淋巴瘤的病因不明，多数与EB病毒的感染有关，90%以上患者EBV检测呈阳性。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>","🧬",{"key":95,"title":96,"html":97,"icon":98,"updatedAt":88},"symptom","症状表现","\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">结外NK\u002FT细胞淋巴瘤的症状和体征包括：\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cp>\u003Cspan style=\"font-size: 15px;\">1、B症状（一般症状）很常见，如淋巴结肿大，脾脏或肝脏肿大，发热、乏力、盗汗、疲劳，以及不明原因的体重减轻等。\u003C\u002Fspan>\u003C\u002Fp>\u003Cp>\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cp>\u003Cspan style=\"font-size: 15px;\">2、肿瘤常局限于鼻部和邻近的结构，其中以鼻咽及颚部最为常见，其次为口咽、咽喉、扁桃体，典型的临床特征是鼻塞，以及持续不愈合的溃疡；鼻外部位好发于皮肤、胃肠道、睾丸、唾液腺等。\u003C\u002Fspan>\u003C\u002Fp>\u003Cp>\u003Cspan style=\"font-size: 15px;\">\u003Cbr\u002F>\u003C\u002Fspan>\u003C\u002Fp>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">3、肿瘤侵犯远处淋巴结或结外器官的情况比较少。\u003C\u002Fspan>\u003C\u002Fdiv>","📋",{"key":100,"title":101,"html":102,"icon":103,"updatedAt":88},"exam","检查方式","\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">根据结外NK\u002FT细胞淋巴瘤的症状和体征，医生可能会进行以下诊断性检查，包括：\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">体格检查：检查患者的一般状况、全身皮肤，以及是否有胸骨压痛，触诊全身浅表淋巴结，是否出现韦氏环（咽淋巴环），并触诊肝脏、脾脏，以及进行体能状态评分。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">血液检查：用于观察不同类型血细胞的数量和形态。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">病理活检：病理活检主要是取多个组织样本在镜下观察，看是否出现ENKTCL的病理学特征（如弥漫性淋巴细胞浸润、血管中心性破坏性生长模式等），其结果对于 ENKTCL 的诊断至关重要。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">CT检查：可用于观察身体各部位的横截面图像，您可能需要接受胸部、腹部以及咽喉、鼻腔等部位的扫描。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">PET\u002FCT检查：可对全身的肿瘤病灶进行显像，并能显示出CT无法充分显像的病变。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">PCR（聚合酶链反应）：用PCR方法可对EB病毒进行定量检测。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">流式细胞分析：该检查可根据细胞的大小、颗粒、抗原表达特征，将细胞分为不同群体，让淋巴瘤的免疫分型一目了然，临床上医生可以根据此项检测做出适合患者自身的治疗方案。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">细胞遗传学方法：主要检测ENKTCL常见染色体数量或结构异常，分子遗传学方法如全外显子测序和靶向测序，主要检测ENKTCL常见的基因突变。这两类检测有助于判断患者的预后并指导治疗。\u003C\u002Fspan>\u003C\u002Fdiv>","🩺",{"key":105,"title":106,"html":107,"icon":108,"updatedAt":88},"treatment","治疗方式","\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">结外NK\u002FT细胞淋巴瘤的治疗主要是放疗、放化疗结合及造血干细胞移植。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">放疗：放疗利用高能射线治疗肿瘤，可单独使用，也可与其他癌症治疗方法联用。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">结外NK\u002FT细胞淋巴瘤对放疗敏感，尤其是处于临床早期的鼻型结外NK\u002FT细胞淋巴瘤，单独进行放疗效果较好。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">化疗：化疗是一种通过口服或注射给药来杀灭肿瘤细胞的药物治疗。化疗药物可以单独使用，也可以与其他化疗药物或其他治疗方法联用。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">对于局限期结外NK\u002FT细胞淋巴瘤，首选以天门冬酰胺酶为基础的方案联合化疗，此外还可以根据患者的情况选择同步放化疗和序贯放化疗。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cbr\u002F>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">造血干细胞移植：医生会先利用大剂量的化疗和放疗来抑制骨髓，随后将采集的健康骨髓干细胞输入患者的血液中，干细胞会随血液循环进入骨骼并重建骨髓。\u003C\u002Fspan>\u003C\u002Fdiv>\u003Cdiv>\u003Cspan style=\"font-size: 15px;\">造血干细胞移植可以作为进展期ENKTCL患者缓解后的首选巩固治疗方法。\u003C\u002Fspan>\u003C\u002Fdiv>","💊",{"author":110,"reviewedBy":110,"lastReviewed":110,"datePublished":110,"citations":111,"disclaimer":110},"",[112,114],{"title":113},"中国淋巴瘤治疗指南（2021年版）",{"title":115},"NCCN Guidelines: T-Cell Lymphomas, Version 1.2021",false,1789384106334]