检索规则说明:AND代表“并且”;OR代表“或者”;NOT代表“不包含”;(注意必须大写,运算符两边需空一格)
检 索 范 例 :范例一: (K=图书馆学 OR K=情报学) AND A=范并思 范例二:J=计算机应用与软件 AND (U=C++ OR U=Basic) NOT M=Visual
作 者:张兴利 罗菁 张佼佼 陈丽 沈杨 易红梅 范立权[3] 糜坚青 Zhang Xingli;Luo Jing;Zhang Jiaojiao;Chen Li;Shen Yang;Yi Hongmei;Fan Liquan;Mi Jianqing(Shanghai Institute of Hematology,State Key Laboratory of Medical Genomics,National Research Center for Translational Medicine at Shanghai,Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine,Shanghai 200025,China;Department of Pathology,Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine,Shanghai 200025,China;Shanghai Institute of Hematology,Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine,Shanghai 200025,China)
机构地区:[1]上海血液学研究所,国家医学基因重点实验室,国家转化医学研究中心,上海交通大学医学院附属瑞金医院血液科,上海200025 [2]上海交通大学医学院附属瑞金医院病理科,上海200025 [3]上海交通大学医学院附属瑞金医院,上海血液病研究所骨髓细胞室,上海200025
出 处:《中华血液学杂志》2022年第12期1028-1033,共6页Chinese Journal of Hematology
基 金:2021年苏州市卫生青年骨干人才"全国导师制"培训项目 (Qngg2021046)。
摘 要:目的探究脾弥漫性红髓小B细胞淋巴瘤(SDRPL)患者的临床特征、疗效及预后。方法回顾性分析2017年5月至2022年4月在上海交通大学医学院附属瑞金医院诊治的8例SDRPL患者的病历资料,对患者的临床特征、实验室检查结果、骨髓和脾脏病理、疗效与预后进行分析及总结。结果8例患者中位年龄54(42~69)岁。确诊时均表现为明显的脾脏肿大,多有淋巴细胞升高,PET/CT显示脾脏代谢不高或轻度增高。临床分期均为Ⅳ期,累及脾脏、外周血和骨髓,未见外周淋巴结受累。肿瘤细胞胞质丰富,易见短毛刺状突起。脾脏病理可见形态均一的小淋巴细胞弥漫浸润脾窦及脾索,白髓萎缩。免疫表型缺乏特异性,多表达CD19、CD20、CD79α等B细胞抗原。中位随访35.5(4~60)个月,脾切除术联合或不联合免疫化疗可使7例患者获得长期生存,1例伴CCND3 P284A和MYC S146L突变的患者在脾切除术后1个月进展为B细胞幼淋巴细胞白血病(B-PLL),在随访16个月时死亡。结论SDRPL患者以中老年为主,临床呈惰性病程。患者大多可获得长期生存,但进展为B-PLL的患者预后较差。Objective To investigate the clinical characteristics,response,and prognosis of splenic diffuse red pulp small B-cell lymphoma(SDRPL).Methods Eight cases of SDRPL were diagnosed and treated at Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine,between May 2017 and April 2022.Data on the clinical features,laboratory results,bone marrow and spleen biopsy results,response,and prognosis were collected and analyzed.Results The median age at diagnosis was 54(42-69)years.Splenomegaly and lymphocytosis were present in all cases,and PET/CT revealed normal to slightly elevated splenic FDG uptake.All cases were in stageⅣ,with spleen,peripheral blood,and bone marrow but no proximal lymph nodes involved.The cytoplasm of neoplastic villous cells was abundant,and splenic pathology showed that small homogenous lymphocytes permeated the splenic sinus and splenic cord,and the white pulp atrophied.Immunohistochemistry was not typical,and B-cell markers including CD19,CD20 and CD79αwere positive.After a median follow up of 35.5(4-60)months,7 cases were alive after splenectomy with or without chemoimmunotherapy.The patient with CCND3 P284A and MYC S146L mutation developed to B-cell prolymphocytic leukemia(B-PLL)1 month after splenectomy and died at 16 months of follow-up.Conclusion A rare indolent B-cell lymphoma that primarily affects the elderly,SDRPL.Most patients achieved long-term survival,but the prognosis of patients who progress to B-PLL was poor.
正在载入数据...
正在载入数据...
正在载入数据...
正在载入数据...
正在载入数据...
正在载入数据...
正在载入数据...
正在链接到云南高校图书馆文献保障联盟下载...
云南高校图书馆联盟文献共享服务平台 版权所有©
您的IP:216.73.216.33