套细胞淋巴瘤349例临床病理学特点及预后

Clinicopathologic features and prognosis of mantle cell lymphoma： an analysis of 349 cases

目的探讨套细胞淋巴瘤（MCL）的临床病理学特点及预后影响因素。方法回顾性分析2004年1月至2016年1月北京友谊医院诊断的349例MCL患者的临床资料，光镜观察肿瘤形态，采用MaxVision^TM－2染色法检测免疫表型，荧光原位杂交技术（FISH）检测IgH/CCND1融合基因，并进行相关统计分析。结果349例MCL患者中，男女比例为2.7∶1.0，中位年龄61岁（范围25～83岁），其中51～70岁者243例（69.6%）。有B症状者占22.4%（70/313）。大部分患者表现为全身浅表淋巴结肿大，临床分期Ⅲ～Ⅳ期占76.1%（235/309）。结外侵犯者占47.9%（148/309），其中最多见的是胃肠，占31.8%（47/148）。脾脏受累占15.4%（47/305）。309例（85.5%）经典型和40例（11.5%）侵袭性变异型均由增生的淋巴样细胞构成。所有病例肿瘤细胞表达CD20和cyclin D1，98.6%（344/349）的病例弱阳性或阳性表达CD5。12例CD5阴性/弱阳性或cyclin D1部分细胞阳性的病例FISH检测结果均阳性。243例（69.6%）获得随访，随访中位时间26个月（3～108个月），患者3年和5年总生存率分别为63.0%和34.8%。单因素分析结果显示年龄〉60岁、脾脏受累、侵袭性变异型、非全网型（依据滤泡树突细胞网与肿瘤细胞的吻合程度≥90%和〈90%将其分为全网型和非全网型）及Ki－67阳性指数〉40%的患者预后差（P〈0.05）。剔除侵袭性变异型后多因素Cox回归模型分析，结果显示年龄、脾脏受累、滤泡树突细胞网与肿瘤细胞吻合度及Ki－67阳性指数是影响MCL患者总生存率的独立预后因素（P〈0.05）。结论MCL以中老年男性多见，患者年龄、脾脏受累、滤泡树突细胞网与肿瘤细胞吻合度及Ki－67阳性指数是MCL预后风险评估的独立预测因子。

ObjectiveTo investigate clinicopathologic features and prognostic factors of mantle cell lymphoma（MCL）.MethodsThe clinical data of 349 MCL patients diagnosed at Beijing Friendship Hospital from January 2004 to January 2016 were retrospectively collected. Corresponding histological sections were reviewed. Additional studies included immunohistochemical staining using the MaxVision two-step method, IgH/CCND1 fusion gene detection by fluorescent in situ hybridization （FISH）, and correlative statistical analysis.ResultsOf 349 patients with MCL, the median patient age was 61 years （range： 25-83 years, M∶F=2.7∶1.0） and the age of 243 patients ranged from 51 to 70 years （69.6%）. Those with B symptoms accounted for 22.4% （70/313）. Most of the patients presented with superficial lymphadenopathy and the clinical stage Ⅲ-Ⅳ accounted for 76.1% （235/309）. Extranodal involvement was seen in 47.9% （148/309）, among which the gastrointestinal tract accounted for 31.8% （47/148） and splenic involvement accounted for 15.4% （47/305）. Three hundred and nine （88.5%） cases were of classical type and 40 （11.5%） cases were of aggressive variant type, and all were composed of proliferating lymphoid cells. All the tumors were positive for CD20 and cyclin D1, and 98.6% （344/349） tumors were weakly positive or positive for CD5. FISH test was positive in 12 cases that were CD5 negative and with cyclin D1 partial expression.Two hundred and forty-three （69.6%） patients had a median follow-up of 26 months （range： 3-108 months）. The 3- and 5-year overall survival rates for patients were 63.0% and 34.8%, respectively. Single factor analysis showed that age of 〉60 years, splenic involvement, aggressive variant type, incompletely overlapping type [Based on the degree of overlap ≥90% and 〈90% between the follicular dendritic cell （FDC） meshwork and tumor cells, the tumors were divided into the completely overlapped type and incompletely overlapped type] and Ki-67 index 〉40% had poor prognosis （P〈0.05）. Multiple factor Cox proportional risk regression analysis after removing the aggressive variant type showed that age, splenic involvement, the degree of overlap between the FDC meshwork and tumor cells and Ki-67 index were independent prognostic factors for overall survival rate of MCL patients （P〈0.05）.ConclusionsMCL is more commonly found among middle-aged and elderly men. Patient age, splenic involvement, degree of overlap between FDC meshwork and tumor cells and Ki-67 index are the independent prognostic indicators for MCL.