原发结外非霍奇金淋巴瘤110例临床分析

Clinical Features of 110 Cases of Primary Extranodal Non-Hodgkin's Lymphoma

目的:探讨原发结外非霍奇金淋巴瘤(PE-NHL)的发病情况、临床特点、诊断及治疗。方法:回顾性分析苏州大学附属第二医院2001年1月～2008年5月收治的PE-NHL 110例。结果:110例PE-NHL占同期收治恶性淋巴瘤患者的60.11%(110/183);原发部位广泛,常见部位依次为胃肠道21.82%(24/110)、Waldeve环10.91%(12/110)、鼻腔和软组织各9.10%(10/110)、纵隔7.27%(8/110)、其他少见部位41.82%(46/110);首发表现以原发器官肿大或肿块为主(77.27%),无特异性;90例患者国际预后指数(IPI)评分:低、中、高危组分别占41.11%、44.44%、14.44%。93例进行免疫分型:B细胞型69.90%,T细胞型30.10%;95例接受治疗,CR 61.05%,PR 16.84%,总有效率77.89%,中位生存时间30个月(2～86个月),5年总生存率27%。24例原发胃肠道非霍奇金淋巴瘤(PGIL),其临床特征与非原发胃肠道组比较无统计学差异,治疗23例,CR 65.21%,PR 17.39%,总有效率82.60%,中位生存时间24个月(2～78个月),5年总生存率30%。结论:本组资料中PE-NHL比例高迭60.11%,超过结内淋巴瘤;其首发部位广泛,临床表现多样但无特异性;全面认识本病、遵循合理的诊治模式是提高疗效的关键。

Objective： To evaluate the incidence, clinical features, diagnosis and treatment of primary extranodal non-Hodgkin＇ s lymphoma （PE-NHL）. Methods： The data of 110 patients diagnosed as PE-NHL between January 2001 and May 2008 were reviewed. Results： These PE-NHL patients counted 60.11% of the 183 malignant lymphoma patients at the same period. The primary sites affected were the gastrointestinal tract 21.82% （24/110）, Waldeyer ring 10.91% （12/110）, nasal cavity 9.10% （10/110）, soft tissue 9.10% （10/ 110）, mediastinum 7.27% （8/110） and other unusual sites 41.82% （46/110）. Symptoms and signs of PE-NHL were not specific, and 77.27% of these cases had a swelling organ or lump of the primary organ affected. According to the International Prognosis Index （IPI）, the percentage of patients in low, intermediate, and high group was 41.11%, 44.44% and 14.44%, respectively. Immunophenotype was assayed in 93 cases. The percentage of B-cell lymphoma was 69.90% while that of T-cell lymphoma was 30.10%. For those 95 cases treated, the effective rate including complete remission （61.05%） and part remission （16.84%） was 77.89%, the median survival was 30 months, and the 5-year overall survival （OS） was 27%. While, for patients with primary gastrointestinal non-Hodgkin＇s lymphomas （PGIL）, the complete remission rate, part remission rate and the effective rate was 65.21%, 17.39% and 82.60%, respectively. The median survival was 24 months, and the 5-year overall survival （OS） was 30%. Conclusion： PE-NHL is more common than nodal lymphoma. The symptoms and signs of PE-NHL of different sites are quite different. To improve the curative strategies of PE-NHL, it is important to make an allround understanding of PE-NHL and follow reasonable mode of diagnosis and therapy.