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淋巴瘤是起源于淋巴结和(或)淋巴组织的一类恶性肿瘤,目前已知的淋巴瘤有近70 种病理类型,分为霍奇金淋巴瘤( Hodgkin's lymphoma,HL)和非霍奇金淋巴瘤( non- Hodgkin's lymphoma,NHL)两大类。自然杀伤/T细胞淋巴瘤(natural killer/T-cell lymphoma,NKTL)是NHL的一种,大多数起源于成熟的自然杀伤(natural killer,NK)细胞,部分起源于细胞毒性T淋巴细胞,几乎均在结外部位生长,故又称结外自然杀伤/T细胞淋巴瘤(extranodal natural killer/T-cell lymphoma,ENKTL)。ENKTL的发病率约占NHL的12%,全部淋巴瘤的6%[1],有明显的地域差异和种族易感性,在亚洲及拉丁美洲人群中的发病率较高[2]。ENKTL可发生于任何年龄,以40~50岁多见[2],其发病与EB病毒(Epstein-Barr virus)感染密切相关[3]。根据累及部位,ENKTL分为3个亚型:鼻型、非鼻型及播散型[3]。其中鼻型是最常见的一种类型,多发生在鼻腔、鼻咽、口咽、咽淋巴环,部分患者的眼眶和副鼻窦也可累及[4],患者常伴有鼻腔或鼻咽出血。偶有病例发生在非鼻部,累及皮肤[5]、胃肠道[6]、睾丸和唾液腺,称为非鼻型。极少数情况下,ENKTL累及骨髓、外周血、肝脾及多个系统,称为播散型NK细胞淋巴瘤或侵袭性NK细胞白血病[4],此类患者常伴发噬血综合征。ENKTL的病理特征是呈血管中心性破坏生长的、弥漫性淋巴细胞浸润,导致组织缺血坏死及黏膜溃疡[3],故ENKTL又称为血管中心性淋巴瘤。与其他类型淋巴瘤相比,ENKTL的侵袭性强、恶性度高,预后极差,尤其是非鼻型和播散型,5年生存率仅为37.9%~49.5%[7]。准确的诊断、分期和及时的疗效评估对ENKTL治疗方案的制定和改善预后至关重要。
目前,以18F-FDG为示踪剂的PET/CT在淋巴瘤中的作用越来越受重视,18F-FDG PET/CT将功能代谢显像和解剖结构显像有机结合,可在淋巴瘤的不同治疗时期测定其代谢相关参数,包括SUVmax、平均标准摄取值(mean standardized uptake value,SUVmean)、肿瘤代谢体积(metabolic tumor volume,MTV)和病灶糖酵解总量(total lesion glycolysis,TLG)等,18F-FDG PET/CT不仅能对淋巴瘤患者进行准确的诊断和分期,而且还可以在患者诊治的各个阶段进行疗效评估及预后预测[8]。笔者就PET/CT在ENKTL诊治中的应用进展进行综述。
PET/CT在结外NK/T细胞淋巴瘤诊治中的应用进展
Application progress of PET/CT in the diagnosis and treatments of extranodal natural killer/T-cell lymphoma
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摘要: 结外自然杀伤/T细胞淋巴瘤(ENKTL)是一种与EB病毒感染相关的非霍奇金淋巴瘤亚型,其侵袭性强,预后不良。准确的诊断和分期、及时的疗效及预后评估对患者治疗策略的制定至关重要。随着18F-FDG PET/CT在淋巴瘤中的广泛应用,其在ENKTL中的价值也越来越受到重视,笔者对PET/CT在ENKTL诊治中的应用进展进行综述。
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关键词:
- 淋巴瘤 /
- 结外NK-T细胞 /
- 正电子发射断层显像术 /
- 体层摄影术,X线计算机 /
- 氟脱氧葡萄糖F18 /
- 诊断 /
- 肿瘤分期 /
- 预后
Abstract: Extranodal natural killer/T-cell lymphoma (ENKTL) is a subtype of non-Hodgkin's lymphoma associated with Epstein-Barr virus infection with high aggressiveness and poor prognosis. Accurate diagnosis and staging, timely curative effect and prognostic evaluation are crucial to the formulation of patient treatment strategies. With the wide application of 18F-FDG PET/CT in lymphoma, its value in ENKTL has also received more and more attention. This article reviews the application progress of PET/CT in the diagnosis and treatment of ENKTL. -
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