中文摘要：

目的 探讨原发乳腺淋巴瘤（PBL）的临床病理、免疫组织化学特征及其与预后的关系.方法 按Ann Arbor临床分期标准及Wiseman和Liao的诊断标准对40例PBL患者进行分期,按照WHO（2008年）造血和淋巴瘤系统肿瘤分类分型,并进行了随访.采用SP法行免疫组织化学染色.结果 （1）40例患者均为女性,中位年龄47岁,右侧20例,左侧16例,双侧4例.临床症状大部分表现为逐渐增大的乳腺无痛性包块,肿块数量＜3个31例 （77.5%,31/40）,≥3个9例（22.5%,9/40）;Ann Arbor分期：33例（82.5%）为Ⅰ～Ⅱ期,7例（17.5%）为Ⅲ～Ⅳ期.9例（24.3%,9/37）血清乳酸脱氢酶（LDH）水平升高;体力状况美国东部肿瘤协作组（ECOG）评分：0～1分34例（85.0%）,2分以上6例（15.0%）;国际预后指数（IPI）评分：29例0～1分（7 8.4%,29/37）,2例2分（5.4%,2/37）,3例3分（8.1%,3/37）,3例4分（8.1%,3/37）.21例（53.8%,21 /39）累及腋窝淋巴结.（2）40例中27例（67.5%）为弥漫性大B细胞淋巴瘤（DLBCL）,8例（20.0%）为黏膜相关淋巴组织结外边缘区B 细胞淋巴瘤（MALT淋巴瘤,1例伴大细胞转化）,2例为滤泡性淋巴瘤（FL,Ⅰ级）,1例为淋巴性浆细胞淋巴瘤,1例为淋巴母细胞性淋巴瘤,1例为外周 T细胞淋巴瘤.40例细胞角蛋白均呈阴性,95.0%（38/40）的肿瘤细胞表达CD20和（或）CD79a;MUM1（57.6%,19/33）、 bcl-6（30.3%,10/33）、bcl-2（72.7%,24/33）呈阳性表达.3例为生发中心B细胞（GCB）亚型,21例为非GCB亚型. （3）37例（92.5%,37/40）获得随访资料,其中23例（62.2%,23/37）存活,14例（37.8%,14/37）死亡.27例 DLBCL的5年总生存率为48.0%,5年无病生存率为36.0%.结论 PBL少见,大多为DLBCL,其中又以非GCB亚型为主.LDH水平、肿块数目、IPI指数为影响预后的独立因素.

英文摘要：

Objective To study the clinicopathologic features and prognosis of primary lymphoma of breast. Methods Forty cases of primary breast lymphoma, diagnosed according to the 2008 World Health Organization classification of hematopoietic and lymphoid tumors, were retrospectively studied.Immunohistochemistry was performed by SP method. The follow-up data were analyzed. Results （ 1 ） All the patients were females and the median age was 47 years. Unilateral and bilateral breast involvement were noted in 36 and 4 patients, respectively. The number of tumor were 31 cases（77. 5% ,31/40） less than 3 ,and 9 cases （22.5% ,9/40） were 3 and more than 3. According to Ann Arbor staging system, 33 cases （82. 5% ） were in stage Ⅰ to Ⅱ and 7 cases （ 17. 5% ） in stage Ⅲ to Ⅳ. The level of LDH in 9 cases （24. 3% ,9/37） went up. For ECOG scores, 34 cases（85.0% ） were 0 to 1 score and 6 cases （ 15.0%）were more than 2 scores. With respect to international prognostic index, 83. 8% （31/37） were of score 0 to 2 and 16. 2% （6/37） were of score 3 and more than 3. The axillary lymph nodes of 21 patients （53. 8%,21/39） were involved by the malignancy. （2） Histologically, 38 cases （95.0% ,38/40） were classified as B-cell lymphoma [including 27 cases （ 67. 5% ） of diffuse large B-cell lymphoma, 8 cases （ 20. 0% ） of mucosa-associated lymphoid tissue lymphoma, 2 eases of follicular lymphoma and 1 case of lymphoplasmacytic lymphoma]. The remaining cases included one case of peripheral T-cell lymphoma and one case of lymphoblastic lymphoma. Immunohistochemically, expression of CD20 ＋/- CD79a were demonstrated in the 38 cases （ 95.0% ） of B-cell lymphoma. The staining for CK was negative in all cases.In 33 cases, the positive rates of MUM-1, bcl-6 and bcl-2 were 57. 6% （ 19/33）, 30.3% （ 10/33 ） and 72. 7% （24/33）, respectively. Three cases were germinal center B cell phenotype and 21 cases were nongerminal center B cell phenotype. （3） Follow-up information was av