Background Primary systemic light chain amyloidosis （AL） is a rare plasma cell disease,our purpose was to analyze the immunophenotypic characteristics of the plasma cells in bone marrow in AL patients,and explore wh...Background Primary systemic light chain amyloidosis （AL） is a rare plasma cell disease,our purpose was to analyze the immunophenotypic characteristics of the plasma cells in bone marrow in AL patients,and explore whether the detection of abnormal plasma cell clones in bone marrow by flow cytometry （FCM） could be used as an important indicator of AL diagnosis.Methods Fresh bone marrow samples were collected from 51 AL,21 multiple myeloma （MM）,and 5 Waldenstr（o）m＇s macroglobulinemia （WM） patients.The immunophenotype of bone marrow cells were analyzed and compared by FCM using a panel of antibodies including CD45,CD38,CD138,CD117,CD56,and CD19.Results In AL,light chain restriction could be identified in 31 cases （60.9%）,in which the λ light chain restriction was found in 24 cases （77.4%）.In MM,κ light chain restriction was found in 13 cases （61.9%）,and λ light chain restriction in eight cases.CD45 on abnormal plasma cells was negative to weakly positive in both AL and MM,but was positive to strongly positive in WM.In the bone marrow plasma cells of the 51 AL,78.4% were CD56＋,68.6% were CD117＋,and 88.2% were CD19-.While in the 21 MM cases,66.7% were CD56＋,38.1% were CD117＋,and 90.4% were CD19-.The plasmacytoid lymphocytes in the five WM patients were CD19＋ and CD56-,CD117-.Conclusion Detection of abnormal plasma cell clones in bone marrow by FCM is valuable for the diagnosis of AL.展开更多
目的:总结并比较意义未明的单克隆丙种球蛋白血症(MGUS)、原发性轻链(pAL)型淀粉样变、多发性骨髓瘤(MM)以及MM继发淀粉样变性患者的临床基线特点,尤其是细胞遗传学异常的差异。方法:回顾性分析和比较2006年2月至2019年8月于中国医学科...目的:总结并比较意义未明的单克隆丙种球蛋白血症(MGUS)、原发性轻链(pAL)型淀粉样变、多发性骨髓瘤(MM)以及MM继发淀粉样变性患者的临床基线特点,尤其是细胞遗传学异常的差异。方法:回顾性分析和比较2006年2月至2019年8月于中国医学科学院血液病医院淋巴瘤诊疗中心就诊,并完善了细胞遗传学异常检测的15例MGUS、34例pAL、842例MM和23例MM继发淀粉样变性患者的临床资料。结果:细胞遗传学统计显示,4组(MGUS vs pAL vs MM vs MM继发淀粉样变性)中t(11;14)易位发生率分别为0%、33.3%、16.4%和15.8%(P=0.037);13q缺失发生率分别为20.0%、14.7%、45.8%和56.5%(P<0.001);1q21扩增发生率分别为50.0%、12.5%、47.4%和40.9%(P=0.001)。31例pAL患者累积发生0个、1个和≥2个细胞遗传学异常(包括13q缺失、17p缺失、1q21扩增和IgH易位)的比例分别为41.9%、41.9%和16.1%;MM患者对应的该比例分别为17.6%、27.3%和55.2%;MM继发淀粉样变性的这一比例与MM相似。亚组分析显示,pAL患者中t(11;14)阴性与阳性两亚组合并其他遗传学异常比例相当(包括13q缺失、17p缺失和1q21扩增);而MM+MGUS患者中t(11;14)阴性病例更易合并13q缺失,且更常同时发生多个遗传学异常。结论:pAL的临床特点,尤其细胞遗传学异常特征与MM及MM继发淀粉样变性具有明显差异。这提示,虽然pAL与MM继发淀粉样变性的起病特征相似,但是二者分属于不同疾病亚型,应仔细加以鉴别。展开更多
Daratumumab（DARA）是首个全人源化IgG1κ型的抗CD38单克隆抗体,现已经在欧美国家上市,用于治疗复发/难治性多发性骨髓瘤（relapsed or refractory multiple myeloma,RRMM）。下面,我们将结合1例复发性MM患者的治疗选择对DARA在浆细胞...Daratumumab（DARA）是首个全人源化IgG1κ型的抗CD38单克隆抗体,现已经在欧美国家上市,用于治疗复发/难治性多发性骨髓瘤（relapsed or refractory multiple myeloma,RRMM）。下面,我们将结合1例复发性MM患者的治疗选择对DARA在浆细胞病治疗中的应用及相关的临床问题进行全面阐述,以提高大家对此类新药的认识。展开更多
基金This work was funded by the National Natural Science Foundation of China （No.81370612） and the Youth Fund Project of the National Natural Science Foundation of China （No.81102211）.
文摘Background Primary systemic light chain amyloidosis （AL） is a rare plasma cell disease,our purpose was to analyze the immunophenotypic characteristics of the plasma cells in bone marrow in AL patients,and explore whether the detection of abnormal plasma cell clones in bone marrow by flow cytometry （FCM） could be used as an important indicator of AL diagnosis.Methods Fresh bone marrow samples were collected from 51 AL,21 multiple myeloma （MM）,and 5 Waldenstr（o）m＇s macroglobulinemia （WM） patients.The immunophenotype of bone marrow cells were analyzed and compared by FCM using a panel of antibodies including CD45,CD38,CD138,CD117,CD56,and CD19.Results In AL,light chain restriction could be identified in 31 cases （60.9%）,in which the λ light chain restriction was found in 24 cases （77.4%）.In MM,κ light chain restriction was found in 13 cases （61.9%）,and λ light chain restriction in eight cases.CD45 on abnormal plasma cells was negative to weakly positive in both AL and MM,but was positive to strongly positive in WM.In the bone marrow plasma cells of the 51 AL,78.4% were CD56＋,68.6% were CD117＋,and 88.2% were CD19-.While in the 21 MM cases,66.7% were CD56＋,38.1% were CD117＋,and 90.4% were CD19-.The plasmacytoid lymphocytes in the five WM patients were CD19＋ and CD56-,CD117-.Conclusion Detection of abnormal plasma cell clones in bone marrow by FCM is valuable for the diagnosis of AL.
文摘目的:总结并比较意义未明的单克隆丙种球蛋白血症(MGUS)、原发性轻链(pAL)型淀粉样变、多发性骨髓瘤(MM)以及MM继发淀粉样变性患者的临床基线特点,尤其是细胞遗传学异常的差异。方法:回顾性分析和比较2006年2月至2019年8月于中国医学科学院血液病医院淋巴瘤诊疗中心就诊,并完善了细胞遗传学异常检测的15例MGUS、34例pAL、842例MM和23例MM继发淀粉样变性患者的临床资料。结果:细胞遗传学统计显示,4组(MGUS vs pAL vs MM vs MM继发淀粉样变性)中t(11;14)易位发生率分别为0%、33.3%、16.4%和15.8%(P=0.037);13q缺失发生率分别为20.0%、14.7%、45.8%和56.5%(P<0.001);1q21扩增发生率分别为50.0%、12.5%、47.4%和40.9%(P=0.001)。31例pAL患者累积发生0个、1个和≥2个细胞遗传学异常(包括13q缺失、17p缺失、1q21扩增和IgH易位)的比例分别为41.9%、41.9%和16.1%;MM患者对应的该比例分别为17.6%、27.3%和55.2%;MM继发淀粉样变性的这一比例与MM相似。亚组分析显示,pAL患者中t(11;14)阴性与阳性两亚组合并其他遗传学异常比例相当(包括13q缺失、17p缺失和1q21扩增);而MM+MGUS患者中t(11;14)阴性病例更易合并13q缺失,且更常同时发生多个遗传学异常。结论:pAL的临床特点,尤其细胞遗传学异常特征与MM及MM继发淀粉样变性具有明显差异。这提示,虽然pAL与MM继发淀粉样变性的起病特征相似,但是二者分属于不同疾病亚型,应仔细加以鉴别。
文摘Daratumumab（DARA）是首个全人源化IgG1κ型的抗CD38单克隆抗体,现已经在欧美国家上市,用于治疗复发/难治性多发性骨髓瘤（relapsed or refractory multiple myeloma,RRMM）。下面,我们将结合1例复发性MM患者的治疗选择对DARA在浆细胞病治疗中的应用及相关的临床问题进行全面阐述,以提高大家对此类新药的认识。
