BACKGROUND Primary light chain amyloidosis is a rare and complex disease with complex clinical features and is highly susceptible to misdiagnosis and underdiagnosis in the early stages.CASE SUMMARY We report a case of...BACKGROUND Primary light chain amyloidosis is a rare and complex disease with complex clinical features and is highly susceptible to misdiagnosis and underdiagnosis in the early stages.CASE SUMMARY We report a case of a 47-year-old female patient whose only initial symptom was periorbital purpura,which was not taken seriously enough.As the disease progressed,pleural effusion gradually appeared,and after systematic diagnosis and treatment,she was diagnosed with“primary light chain amyloidosis”.She achieved rapid hematological remission after treatment with a daratumumab+bortezomib+cyclophosphamide+dexamethasone regimen.CONCLUSION Periorbital purpura can be the only initial symptom of primary light chain amyloidosis;we should pay attention to the cases where the initial clinical symptoms are only periorbital purpura.展开更多
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
目的探讨根据国际骨髓瘤工作组(International Myeloma Working Group,IMWG)标准不同浆细胞病分型患者骨髓浆细胞数量及免疫表型的差异。方法回顾性分析2019年6月12日至2023年9月5日在复旦大学附属中山医院厦门医院诊治的浆细胞病患者...目的探讨根据国际骨髓瘤工作组(International Myeloma Working Group,IMWG)标准不同浆细胞病分型患者骨髓浆细胞数量及免疫表型的差异。方法回顾性分析2019年6月12日至2023年9月5日在复旦大学附属中山医院厦门医院诊治的浆细胞病患者血清学及骨髓流式结果。结果纳入102例浆细胞病患者,男性63例,女性39例,发病年龄22~85岁,其中意义未明的单克隆丙种球蛋白血症46例,冒烟型骨髓瘤5例,多发性骨髓瘤39例,轻链淀粉样变性12例。所有患者均伴有M蛋白,包含IgG型58例、非IgG型44例。所有患者骨髓均可检测到浆细胞,其中79例患者骨髓检测到异常浆细胞,63例患者骨髓检测到正常浆细胞,40例患者骨髓同时检测到正常及异常浆细胞。52例患者骨髓的异常浆细胞表达CD56,12例患者骨髓异常浆细胞表达CD117。不同疾病组间的性别、年龄差异无统计学意义。不同疾病组间的M蛋白类型及浓度、血清受累/非受累游离轻链、骨髓总浆细胞(包括正常及异常浆细胞)/有核细胞、骨髓异常浆细胞/有核细胞、骨髓异常浆细胞/骨髓总浆细胞、骨髓正常浆细胞/骨髓总浆细胞的差异均有统计学意义(P均<0.05)。不同疾病组中异常浆细胞CD56的表达存在统计学差异(P=0.009),而CD117的表达差异无统计学意义。结论轻链淀粉样变性患者骨髓克隆性浆细胞的比例、克隆性浆细胞/骨髓总浆细胞、异常浆细胞CD56表达比例与意义未明单克隆丙种球蛋白血症相仿,而与多发性骨髓瘤患者有显著差异。展开更多
基金Supported by the Henan Province Medical Science and Technology Research Plan Joint Construction Project,No.LHGJ20210533Xinxiang Science and Technology Research Project,No.GG2020029.
文摘BACKGROUND Primary light chain amyloidosis is a rare and complex disease with complex clinical features and is highly susceptible to misdiagnosis and underdiagnosis in the early stages.CASE SUMMARY We report a case of a 47-year-old female patient whose only initial symptom was periorbital purpura,which was not taken seriously enough.As the disease progressed,pleural effusion gradually appeared,and after systematic diagnosis and treatment,she was diagnosed with“primary light chain amyloidosis”.She achieved rapid hematological remission after treatment with a daratumumab+bortezomib+cyclophosphamide+dexamethasone regimen.CONCLUSION Periorbital purpura can be the only initial symptom of primary light chain amyloidosis;we should pay attention to the cases where the initial clinical symptoms are only periorbital purpura.
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.
文摘目的探讨根据国际骨髓瘤工作组(International Myeloma Working Group,IMWG)标准不同浆细胞病分型患者骨髓浆细胞数量及免疫表型的差异。方法回顾性分析2019年6月12日至2023年9月5日在复旦大学附属中山医院厦门医院诊治的浆细胞病患者血清学及骨髓流式结果。结果纳入102例浆细胞病患者,男性63例,女性39例,发病年龄22~85岁,其中意义未明的单克隆丙种球蛋白血症46例,冒烟型骨髓瘤5例,多发性骨髓瘤39例,轻链淀粉样变性12例。所有患者均伴有M蛋白,包含IgG型58例、非IgG型44例。所有患者骨髓均可检测到浆细胞,其中79例患者骨髓检测到异常浆细胞,63例患者骨髓检测到正常浆细胞,40例患者骨髓同时检测到正常及异常浆细胞。52例患者骨髓的异常浆细胞表达CD56,12例患者骨髓异常浆细胞表达CD117。不同疾病组间的性别、年龄差异无统计学意义。不同疾病组间的M蛋白类型及浓度、血清受累/非受累游离轻链、骨髓总浆细胞(包括正常及异常浆细胞)/有核细胞、骨髓异常浆细胞/有核细胞、骨髓异常浆细胞/骨髓总浆细胞、骨髓正常浆细胞/骨髓总浆细胞的差异均有统计学意义(P均<0.05)。不同疾病组中异常浆细胞CD56的表达存在统计学差异(P=0.009),而CD117的表达差异无统计学意义。结论轻链淀粉样变性患者骨髓克隆性浆细胞的比例、克隆性浆细胞/骨髓总浆细胞、异常浆细胞CD56表达比例与意义未明单克隆丙种球蛋白血症相仿,而与多发性骨髓瘤患者有显著差异。
