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意义未明的单克隆丙种球蛋白病患者的临床特征及进展风险因素

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

作者信息

Guan A, Shen K N, Zhang L, Cao X X, Su W, Zhou D B, Li J

机构信息

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.

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2023 Feb 14;44(2):137-140. doi: 10.3760/cma.j.issn.0253-2727.2023.02.009.

Abstract

To analyze the clinical presentation and progression risk factors of patients with monoclonal gammopathy of undetermined significance (MGUS) in China. We retrospectively assessed the clinical features and disease progression of 1 037 patients with monoclonal gammopathy of undetermined significance between January 2004 and January 2022 at Peking Union Medical College Hospital. A total of 1 037 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 0.99/100 person-years, =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 (=0.005) . 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.

摘要

分析中国意义未明单克隆丙种球蛋白病(MGUS)患者的临床表现及疾病进展风险因素。我们回顾性评估了2004年1月至2022年1月在北京协和医院就诊的1037例意义未明单克隆丙种球蛋白病患者的临床特征及疾病进展情况。本研究共纳入1037例患者,其中男性636例(63.6%),中位年龄58(18 - 94)岁。血清单克隆蛋白的中位浓度为2.7(0 - 29.4)g/L。单克隆免疫球蛋白类型为IgG的患者有380例(59.7%),IgA的患者有143例(22.5%),IgM的患者有103例(16.2%),IgD的患者有4例(0.6%),轻链型的患者有6例(0.9%)。171例(31.9%)患者血清游离轻链比值(sFLCr)异常。根据梅奥诊所疾病进展风险模型,低风险、中低风险、中高风险和高风险组患者的比例分别为254例(59.5%)、126例(29.5%)、43例(10.1%)和4例(0.9%)。中位随访时间为47(1 - 204)个月,795例患者中有34例(4.3%)疾病进展,22例(2.8%)死亡。总体疾病进展率为1.06(0.99 - 1.13)/100人年。非IgM型MGUS患者每100人年的疾病进展率显著高于IgM型MGUS患者(2.87/100人年对0.99/100人年,P = 0.002)。梅奥分类中低风险、中低风险和中高风险组的非IgM型MGUS患者每100人年的疾病进展率分别为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)。与非IgM型MGUS相比,IgM型MGUS有更高的疾病进展风险。梅奥诊所疾病进展风险模型适用于中国的非IgM型MGUS患者。

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