原发性乳腺弥漫大B细胞淋巴瘤的临床病理特征及预后

A retrospective analysis of clinical characteristics and prognostic factors for primary breast diffuse large B cell lymphoma

目的探讨原发性乳腺弥漫大B细胞淋巴瘤(primary breast diffuse large B-cell lymphoma,PBDLBCL)的临床特点、免疫表型及预后。方法回顾性分析2006年1月~2016年12月49例PBDLBCL的临床病理资料及免疫组化En Vision法染色结果。结果 49例患者女性47例,男性2例,年龄24~79岁,中位年龄48岁;镜下见肿瘤细胞中等大小或偏大,弥漫一致浸润分布于乳腺小叶间、导管周围、间质及脂肪组织内,部分呈单行条索状排列。免疫表型:37例为非生发中心型,12例为生发中心型;Ki-67增殖指数均大于40%。Ann Arbor分期:ⅠEA期16例,ⅡEA期28例,ⅣE期5例;IPI评分:30例0~1分,10例2分,9例3分。随访5~146个月,3年患者总生存期为51.2%,5年为36.7%,单因素分析结果显示:临床分期、LDH水平、IPI评分、BCL-2蛋白表达、BCL-6蛋白表达的患者3、5年生存期,差异有统计学意义;Cox回归多因素分析显示IPI危险分级增加是PBDLBCL独立的不良预后因素。结论 PBDLBCL诊断主要依靠病理活检及免疫表型,免疫表型以非生发中心为主,治疗以手术、放、化疗等综合治疗方案为宜。预后需多因素综合评价,IPI危险分级增加是PBDLBCL独立的不良预后因素。

Purpose To study the clinical features, immu- nophenotypes and prognostic factors of primary breast diffuse large B-celllymphoma （PBDLBCL）. Methods The clinical pathological data of 49 cases of PBDLBCL during January 2006 to December 2016 were retrospectively analysed, and the basic clinical and pathologic data, pathologic types and the immuno- histochemical slides by EnVision method for staining were sum- marized. Results 47 cases were women and 2 cases were men. The age ranged from 24 to 79 year old with the median age of 48 year old. On microscopic observation, tumor cells were large to medium-sized which characterized as diffuse infiltration between the lobules of mammary gland, around the duct, interstitial and fat tissue, some were single file cord pattern. The immunopheno- type showed 37 cases were of non-GCB, 12 cases were GCB type. Ki-67 index were greater than 40%. According to Ann Ar- bor staging, 16 cases were stage I EA, 28 cases were stage Ⅱ EA, 5 cases were stage IVE. IPI score： 30 cases with 0 - 1 score, 10 cases with 2 score, 9 cases with 3 score. Patients were followed up from 5 to 146 months, The 3-year overall sur- vival （OS） rate was 51.2% and 5-year OS rate was 36. 7%. Single factor analysis showed that there were statistically signifi- cant difference in clinical stage, levels of LDH, IPI score, BCL- 2 protein expression, and BCL-6 protein expression in 3 and 5 years of OS rate. The multiple factor analysis of Cox regression showed that the increase of IPI risk classification was the inde- pendent adverse prognostic factor of primary breast diffuse large B-cell lymphoma. Conclusion The diagnosis of PBDLBCL is confirmed by pathological biopsy and immunohistochemical markers. The immunophenotype was mainly non-GCB type. Comprehensive treatment including surgery, chemotherapy and radiotherapy is appropriate. The prognosis should be comprehen- sively evaluated by multiple factors. IPI increase risk classifica- tion is the independent adverse prognostic factor.