我国血管内大B细胞淋巴瘤的临床亚型、诊治及预后分析  

Clinical subtypes,diagnosis,treatment and prognosis of intravascular large B-cell lymphoma in China

作　　者：陈佳梅 罗波[2] 罗茜茜 蔡高科 董作坤 石薇 陈永顺 CHEN Jiamei;LUO Bo;LUO Xixi;CAI Gaoke;DONG Zuokun;SHI Wei;CHEN Yongshun(Center of Oncology,Renmin Hospital of Wuhan University,Wuhan 430060,China;Department of Pathology,the Central Hospital of Wuhan,Tongji Medical College,Huazhong University of Science and Technology,Wuhan 430014,China;Department of General Family Medicine,Shanghai Fifth People′s Hospital,Fudan University,Shanghai 200240,China)

机构地区：[1]武汉大学人民医院肿瘤中心,武汉大学第一临床学院,武汉430060 [2]华中科技大学同济医学院附属武汉市中心医院病理科,武汉430014 [3]复旦大学附属上海市第五人民医院全科医学科,上海200240

出　　处：《中国癌症防治杂志》2021年第3期288-294,共7页CHINESE JOURNAL OF ONCOLOGY PREVENTION AND TREATMENT

基　　金：国家自然科学基金项目(81901820);中央高校基本科研业务费专项资金资助项目(2042019kf0063);湖北省卫生健康科研基金项目(WJ2019H365);武汉市卫生健康科研基金项目(WX19Q18)。

摘　　要：目的探讨我国血管内大B细胞淋巴瘤(intravascular large B-cell lymphoma,IVLBCL)不同亚型间临床表现、诊治及预后差异。方法系统检索并回顾性分析我国1994—2021年文献报道的185例IVLBCL。结果185例IVLBCL患者中共活检确诊178例,其中61.8%(81/131)有2个或2个以上器官受累。首发症状与辅助检查结果高度异质性。中枢神经系统(CNS)受累型35例,噬血细胞综合征相关型(HPS)29例,其他78例,未知43例。相比HPS型,CNS受累型不明原因发热发生率(25.7%vs 86.2%,P<0.001),贫血/血小板减少发生率(50.0%vs 100.0%,P=0.004),骨髓侵犯发生率(21.4%vs93.1%,P<0.001)以及接受化疗比例(40.0%vs 84.0%,P=0.001)较低。CNS受累型预后差于HPS相关型(2.0个月vs未到达,P<0.001)及其他型(2.0个月vs 23.0个月,P<0.001)。化疗组较非化疗组1年生存率升高(53.4%vs 2.7%,P<0.001)。结论我国IVLBCL临床表现具有高度异质性,CNS受累型预后较HPS相关型差,化疗可显著改善预后。Objective To investigate the differences in clinical manifestations,diagnosis,treatment and prognosis among different subtypes of intravascular large B-cell lymphoma(IVLBCL)in China.Methods A total of 185 cases of IVLBCL which were reported in the literature from 1994 to 2021 in China were systematically retrieved and retrospectively analyzed.Results A total of 178 cases of 185 IVLBCL patients were diagnosed by biopsy,of which 61.8%(81/131)had two or more organs involved.Initial symptoms and ancillary examination results were highly diversified.Among all these cases,35 were central nervous system(CNS)involvement,29 hemophagocytic syndrome(HPS)involvement,78 other involvement,and 43 unknown.Compared with the HPS involvement,the CNS involement had lower incidence rates of fever(25.7%vs 86.2%,P<0.001),anemia/thrombocytopenia(50.0%vs 100.0%,P=0.004),bone marrow invasion(21.4%vs 93.1%,P<0.001),and the rate of receiving chemotherapy(40.0%vs 84.0%,P=0.001).The prognosis of the CNS involvement was worse than that of the HPS involvement(2.0 months vs not reached,P<0.001)and others(2.0 months vs 23.0 months,P<0.001).The 1-year survival rate of the chemotherapy group was higher than that of the non-chemotherapy group(53.4%vs 2.7%,P<0.001).Conclusions The clinical manifestation of IVLBCL in China is highly diversified.The prognosis of the CNS involvement is worse than that of the HPS involvement,and chemotherapy can significantly improve the prognosis.

关 键 词：血管内淋巴瘤 大B细胞 临床分型 中枢神经系统淋巴瘤 

分 类 号：R733.4[医药卫生—肿瘤]