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作 者:刘咏 尤红英 颜灵芝[1] 金松[1] 商京晶[1] 施晓兰[1] 颜霜[1] 姚卫芹 吴德沛[1] 刘蔚[2] 傅琤琤[1] Liu Yong;You Hongying;Yan Lingzhi;Jin Song;Shang Jingjing;Shi Xiaolan;Yan Shuang;Yao Weiqin;Wu Depei;Liu Wei;Fu Chengcheng(Jiangsu Institute of Hematology,National Clinical Research Center for Hematologic Diseases,NHC Key Laboratory of Thrombosis and Hemostasis,The First Affiliated Hospital of Soochow University,Suzhou 215000,China;Department of Pathology,The First Affiliated Hospital of Soochow University,Suzhou 215000,China)
机构地区:[1]苏州大学附属第一医院,江苏省血液研究所,国家血液系统疾病临床医学研究中心,国家卫生健康委员会血栓及止血重点研究室,苏州215000 [2]苏州大学附属第一医院病理科,苏州215000
出 处:《中华血液学杂志》2022年第4期330-335,共6页Chinese Journal of Hematology
摘 要:目的分析合并轻链型淀粉样变性(AL)的初诊多发性骨髓瘤(MM)患者的临床特征、疗效及预后。方法回顾性分析苏州大学附属第一医院自2017年1月1日至2018年10月31日收治的160例初诊MM患者的临床资料。根据患者骨髓、皮肤和其他部位组织病理活检结果是否合并淀粉样变性分为两组,即合并淀粉样变性的MM(MM+AL)组和MM组,比较两组患者的临床特征及疗效。结果160例初诊MM患者中,MM+AL组42例,MM组118例。在临床特征方面,MM+AL组的受累轻链与非受累轻链差值(dFLC)明显高于MM组(P=0.039)。诱导治疗后MM+AL组较MM组有更高的总缓解率(85.7%对79.7%,P<0.05)和非常好的部分缓解率(76.2%对55.1%,P<0.05)。中位随访26(0.25~41)个月,MM+AL组与MM组患者总生存(OS)和无进展生存的差异无统计学意义(P值均>0.05),自体造血干细胞移植组患者的OS优于未移植组患者(P值均<0.05)。MM+AL组心脏受累患者的预后较MM组和MM+AL组非心脏受累患者差(P<0.001),中位OS时间仅为13个月。结论MM+AL患者和MM患者的鉴别诊断需行组织病理活检,尤其是dFLC明显增高患者。MM+AL组患者4个疗程诱导化疗后总体缓解率较MM组更高。MM+AL组累及心脏患者预后较差。Objective To analyze the clinical characteristics,treatment response,and prognosis of newly diagnosed symptomatic multiple myeloma(MM)patients with systemic light chain amyloidosis(AL).Methods The clinical data of 160 patients with newly diagnosed MM treated at the First Affiliated Hospital of Soochow University from January 1,2017 to October 31,2018,were retrospectively analyzed.According to the histopathological biopsy results of bone marrow,skin,and other tissues,the patients were divided into two groups according to whether amyloidosis was combined or not,namely,the MM+AL group and the MM group.The clinical characteristics and treatment responses of the two groups were compared.Results Among the 160 patients with newly diagnosed MM,there were 42 cases in the MM+AL group and 118 cases in the MM group.In terms of clinical features,the involved light chain and noninvolved light chain(dFLC)in the MM+AL group was significantly higher than that in the MM group(P=0.039).After induction treatment,the MM+AL group had a higher overall response rate(85.7%vs 79.7%,P<0.05)and higher excellent partial response(76.2%vs 55.1%,P<0.05).After a median follow-up of 26(0.25–41)months,there was no significant difference in the progression free survival and overall survival(OS)between the two groups(P>0.05).The OS of patients in autologous hematopoietic stem cell transplantation group was better than that in non transplantation group(P<0.05).The prognosis of patients with cardiac involvement in the MM+AL group was significantly worse than that in the MM group and MM+AL group without cardiac involvement(P<0.001),with a median OS of only 13 months.Conclusion The differential diagnosis between the MM+AL and MM groups requires histopathology,particularly for patients with significantly increased dFLC.The overall remission rate of patients in MM+AL group after 4 courses of induction chemotherapy was higher than that in MM group.The prognosis of patients with cardiac involvement in MM+AL group was poor.
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