摘要
目的:分析IgM型系统性轻链型(IgM⁃AL)淀粉样变性的临床特征、治疗和预后。方法:回顾性分析2010年8月至2020年12月确诊的17例IgM⁃AL淀粉样变性患者的一般情况、临床表现和实验室检查特点,治疗反应及预后。结果:患者平均年龄61岁(49~75岁),均有肾脏受累,8例(47%)患者合并心脏受累。血清免疫固定电泳13例为λ⁃IgM,4例为κ⁃IgM,15例(88.2%)血清IgM浓度异常升高。与IgG⁃AL淀粉样变性患者相比,IgM⁃AL淀粉样变性患者淋巴结受累更多(17.6%vs 3.0%,P=0.018),骨髓浆细胞比例更低(1.0%vs 3.2%,P<0.001),浆细胞间期荧光原位杂交(iFISH)检测阳性率更低(14.3%vs 65.3%,P=0.031),尿视黄醇结合蛋白(RBP)浓度更低(0.3mg/L vs 0.6mg/L,P=0.026),血清IgM浓度更高(7.97g/L vs 0.79g/L,P<0.001)。经化疗和(或)大剂量美法仑/自体造血干细胞移植后,IgM⁃AL淀粉样变性患者总体血液学缓解率66.7%,深度血液学缓解率为33.3%,但完全缓解率低(8.3%)。患者2年生存率76%,心脏受累严重的患者预后不佳。结论:IgM⁃AL淀粉样变性多为λ轻链型,淋巴结受累比例较高,骨髓浆细胞负荷低且遗传学改变轻,大多数患者血清IgM浓度异常升高。抗浆细胞治疗有效,血液学完全缓解率低。心脏受累的严重程度是影响预后的主要因素。
Objective:To analyze the clinical features and prognosis of systemic light chain amyloidosis associated with Immunoglobulin M(IgM⁃AL).Methodology:We retrospectively analyzed the general situation,clinical manifestations,laboratory examination features,treatment responses and prognosis of 17 patients with IgM⁃AL.Results:The median age was 61(49~75)years,17 patients(100%)had renal involvement and 8 patients(47%)had cardiac involvement.Serum immunofixation electrophoresis showed that 13 cases wereλ⁃IgM,4 cases wereκ⁃IgM,and 15 cases(88.2%)had elevated serum IgM concentration.Comparied with IgG⁃AL patients,IgM⁃AL had more lymph node involvement(17.6%vs 3%,P=0.018),lower percentage of bone marrow plasma cells(1.0%vs 3.2%,P<0.001),lower abnormal rate of plasma cell interphase florescence in situ hybridization(14.3%vs 65.3%,P=0.031),lower urinary retinol binding protein(0.3 mg/L vs 0.6 mg/L,P=0.026),and higher serum IgM(7.97 g/L vs 0.79 g/L,P<0.001).After chemotherapy and/or high dose melphalan and autologous stem cell transplantation,the overall hematologic response rate was 66.7%and the deep hematologic response rate was 33.3%,but the complete response rate was low(8.3%).The 2⁃year survival rate of patients was 76%.Patients with severe heart involvement had poor prognosis.Conclusion:λlight chain abnormal was more common in IgM⁃AL,with higher lymph nodes involvemen,lower load of bone marrow plasma cells and genetic changes when compared with IgG⁃AL.Anti⁃plasma cell therapy is effective,but complete hematologic response rate was low.The severity of cardiac involvement is a major prognostic factor.
作者
缪福佳
任贵生
郭锦洲
赵亮
徐维玮
黄湘华
MIAO Fujia;REN Guisheng;GUO Jinzhou;ZHAO Liang;XU Weiwei;HUANG Xianghua(National Clinical Research Center of Kidney Diseases,Nanjing University School of Medicine,Jinling Hospital,Nanjing 210016,China)
出处
《肾脏病与透析肾移植杂志》
CAS
CSCD
北大核心
2021年第5期401-406,共6页
Chinese Journal of Nephrology,Dialysis & Transplantation
基金
江苏省重点研发计划社会发展面上项目(BE2017721)
江苏省青年医学重点人才(QNRC2016895)。
