112例原发性韦氏环淋巴瘤的临床病理特征及预后分析

Analysis of Clinicopathological Characteristics and Prognosis of 112 Patients with Primary Waldeyer's Ring Lymphoma

目的:探讨原发性韦氏环淋巴瘤(primary Waldeyer's ring lymphoma,PWRL)的临床病理特征并分析其疗效和预后因素。方法:回顾性分析天津医科大学肿瘤医院2009年1月至2014年1月收治的经病理和免疫组织化学确诊的112例PWRL的临床资料。结果:PWRL占同期淋巴瘤的3.9%(112/2890),中位发病年龄为51.5岁。PWRL的首发部位常见于扁桃体、鼻咽、舌根和口咽,所占比例分别为63.4%(71/112)、22.3%(25/112)、5.3%(6/112)及4.5%(5/112)。PWRL病理类型以弥漫性大B细胞淋巴瘤(DLBCL)(58%)和结外NK/T细胞淋巴瘤(NKTCL)(15.2%)最常见。PWRL患者治疗总体反应率为82.2%,CR为51.4%,PR为30.8%,5年总生存率(OS)为71.6%。韦氏环B细胞淋巴瘤(B-PWRL)患者采用化疗联合美罗华组,5年OS率达94.7%;化疗联合放疗能够明显改善韦氏环T细胞淋巴瘤(T-PWRL)患者预后,5年OS率为88.9%。年龄、分期、病理表型、IPI评分、血清乳酸脱氢酶(LDH)水平、β2微球蛋白水平及初治疗效为预后相关因子。多因素分析显示,年龄大于60岁、LDH水平、病理表型及初治疗效是影响患者总生存的独立危险因素。结论:PWRL预后较好,其病理类型直接影响预后,且对治疗有指导意义。PWRL的治疗倾向于综合治疗,对T-PWRL应采用放化疗联合方案,而对于B-PWRL联合美罗华化疗更优,初治疗效对患者的预后至关重要。年龄、LDH水平也是影响预后的重要因素。

Objective: To investigate the clinical and pathological characteristics of patients with primary Waldeyer's ring lymphomas( PWRL),and to analyze its therapeutic efficacy and prognostic factors. Methods: A total of 112 patients with PWRL confirmed by pathological and immunohistochemical methods between January 2009 and January 2014 were studied. Clinical data were collected and analyzed retrospectively. Results: PWRL accounted for 3. 9% of lymphoma over the same period. Median age of patients with PWRL was 51. 5 years old. The affected areas were tonsil,nasopharynx,tongue base and oropharynx,which accounted for 63. 4%( 71 /112),22. 3%( 25 /112),5. 3%( 6 /112) and4. 5%( 5 /112) respectively. The most common pathological types of these four areas were diffused large B-cell lymphoma( DLBCL) and extranodal NK /T cell lymphoma( NKTCL) which accounted for 58% and 15. 2%. The overall response rate( CR/CRu = 51. 4%; PR = 30. 8%) in all patients was 82. 2%,the estimated 5-year overall survival( OS)rate were 71. 6%. The 5-year OS rate were 94. 7% in the group used Rituximab. Meanwhile,chemotherapy combined with radiotherapy could improve the outcome of T-cell PWRL patients and the 5-year OS rate were 88. 9%. Age,disease stages,pathological types,IPI scores,LDH level,β2-MG level and the efficacy of initial therapy were prognostic factors with statistical significance. Cox multivariate analysis showed that age of more than 60 years,LDH level,pathological types and the efficacy of the initial therapy were independently associated with OS. Conclusion: PWRL has a relatively good prognosis. The pathological types affect the prognosis directly and guide treatment. Combined modality therapy should be chosen for patients with PWRL. Patients with T-cell PWRL should accept chemotherapy combined with radiotherapy,while rituximab may be better for B-cell PWRL. The efficacy of initial therapy is crucial for the outcome of patients. Age and LDH level are also important prognostic factors.