意义未明的单克隆丙种球蛋白血症的临床特征及疾病进展相关因素分析  

Clinical characteristics and progression risk factors for patients with monoclonal gammopathy of undetermined significance

作　　者：关艾 沈恺妮 张路[1] 曹欣欣[1] 苏薇[2] 周道斌[1] 李剑[1] Guan Ai;Shen Kaini;Zhang Lu;Cao Xinxin;Su Wei;Zhou Daobin;Li Jian(Department of Hematology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;Department of Clinical Laboratory,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China)

机构地区：[1]中国医学科学院、北京协和医学院北京协和医院血液内科,北京100730 [2]中国医学科学院、北京协和医学院北京协和医院检验科,北京100730

出　　处：《中华血液学杂志》2023年第2期137-140,共4页Chinese Journal of Hematology

摘　　要：目的探究我国意义未明的单克隆丙种球蛋白血症(MGUS)患者的临床特征及疾病进展相关因素。方法回顾性分析2004年1月至2022年1月北京协和医院确诊的1037例MGUS患者的临床资料。结果1037例患者中,男636例(61.3%),中位诊断年龄58(18~94)岁,中位血清单克隆免疫球蛋白(M蛋白)水平为2.7(0~29.4)g/L。IgG型、IgA型、IgM型、IgD型、轻链型分别为380例(59.7%)、143例(22.5%)、103例(16.2%)、4例(0.6%)、6例(0.9%)。血清游离轻链比例(sFLCr)异常患者171例(31.9%)。按照梅奥危险分层,低危、中低危、中高危、高危组患者分别为254例(59.5%)、126例(29.5%)、43例(10.1%)、4例(0.9%)。中位随访47(1~204)个月后,34例(4.3%)发生疾病进展,22例(2.8%)死亡,进展率为1.06(0.99~1.13)/100人年。IgM-MGUS的进展率显著高于非IgM-MGUS(2.87/100人年对0.99/100人年,P=0.002)。在非IgM-MGUS组患者中,梅奥分层为低危、中低危、中高危组患者的进展率依次为0.32(0.25~0.39)/100人年、1.82(1.55~2.09)/100人年和2.71(1.93~3.49)/100人年,差异有统计学意义(P=0.005)。结论与非IgM-MGUS相比,IgM-MGUS的疾病进展风险更高。梅奥危险分层模型适用于中国非IgM-MGUS患者。Objective To analyze the clinical presentation and progression risk factors of patients with monoclonal gammopathy of undetermined significance(MGUS)in China.Methods We retrospectively assessed the clinical features and disease progression of 1037 patients with monoclonal gammopathy of undetermined significance between January 2004 and January 2022 at Peking Union Medical College Hospital.Results A total of 1037 patients were recruited in the study,including 636 males(63.6%),with a median age of 58(18-94)years.The median concentration of serum monoclonal protein was 2.7(0-29.4)g/L.The monoclonal immunoglobulin type was IgG in 380 patients(59.7%),IgA in 143 patients(22.5%),IgM in 103 patients(16.2%),IgD in 4 patients(0.6%),and light chain in 6 patients(0.9%).171 patients(31.9%)had an abnormal serum-free light chain ratio(sFLCr).According to the Mayo Clinic model for risk of progression,the proportion of patients in the low-risk,medium-low-risk,medium-high risk,and high-risk groups were 254(59.5%),126(29.5%),43(10.1%),and 4(0.9%),respectively.With a median follow-up of 47(1-204)months,34 of 795 patients(4.3%)had disease progression,and 22(2.8%)died.The overall progression rate was 1.06(0.99-1.13)/100 person-years.Patients with non-IgM MGUS have a markedly higher disease progression rate per 100 person-years than IgM-MGUS(2.87/100 person-years vs 0.99/100 person-years,P=0.002).The disease progression rate per 100 person-years in non-IgM-MGUS patients of Mayo classification low-risk,medium-low risk and medium-high risk groups were 0.32(0.25-0.39)/100 person-years,1.82(1.55-2.09)/100 person-years,and2.71(1.93-3.49)/100 person-years,which had statistically difference(P=0.005).Conclusion In comparison to non-IgM-MGUS,IgM-MGUS has a greater risk of disease progression.The Mayo Clinic progression risk model applies to non-IgM-MGUS patients in China.

关 键 词：意义未明单克隆丙种球蛋白病 疾病恶化 

分 类 号：R55[医药卫生—血液循环系统疾病]