Cao Xin-Xin, Meng Qi, Mao Yue-Ying, Su Wei, Zhen Jun-Feng, Shen Kai-Ni, Zhang Chun-Lan, Huang Xu-Fei, Duan Ming-Hui, Zhang Wei, Zhu Tie-Nan, Cai Hua-Cong, Chen Miao, Zhou Dao-Bin, Li Jian
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
Department of Laboratory Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
Leuk Res. 2016 Jul;46:85-8. doi: 10.1016/j.leukres.2016.05.002. Epub 2016 May 4.
We retrospectively evaluated the clinical features, serum levels of IgM, and prevalence of IgM related diseases in patients with serum immunofixation electrophoresis (sIFE) confirmed IgM monoclonal gammopathy at our center.
We included patients with sIFE confirmed IgM monoclonal gammopathy between January 2008 and December 2014 in this retrospective study. We evaluated clinical data, sIFE, serum IgM levels, and diagnosis.
In total, 7107 patients had sIFE confirmed monoclonal gammopathy, with 377 (5.3%) patients having the IgM type. The median age was 62 years (range, 19-105 years). The median level of serum IgM is 8.3g/L (range, 0.24-150g/L). The diagnosis included monoclonal gammopathy of undetermined significance (MGUS, 157 patients, 41.6%), Waldenstrom macroglobulinemia (WM, 105 patients, 27.9%), B cell non-Hodgkin's lymphoma (69 patients, 18.3%), primary cold agglutinin disease (pCAD, 16 patients, 4.2%), primary amyloidosis (14 patients, 3.7%), cryoglobulinaemia (six patients, 1.6%), IgM MGUS associated neuropathy (five patients, 1.3%), multiple myeloma (three patients, 0.8%), and POEMS syndrome (two patients, 0.5%). Levels of serum IgM>15.5g/L were 80.6% sensitive and 89.2% specific for the diagnosis of WM. Kappa type light chain indicated the diagnosis of WM, pCAD, IgM MGUS associated neuropathy and cryoglobulinaemia, while lambda type light chain indicated POEMS and amyloidosis. There were 41/157 (26.1%) MGUS patients diagnosed with complications due to IgM-unrelated autoimmune diseases.
IgM monoclonal gammopathy contains a broad spectrum of diseases. Levels of serum IgM and the type of light chain can be used to help with differential diagnosis. The association between MGUS and some autoimmune diseases requires further investigation.
我们回顾性评估了在本中心经血清免疫固定电泳(sIFE)确诊为IgM单克隆丙种球蛋白病的患者的临床特征、血清IgM水平及IgM相关疾病的患病率。
在这项回顾性研究中,我们纳入了2008年1月至2014年12月期间经sIFE确诊为IgM单克隆丙种球蛋白病的患者。我们评估了临床数据、sIFE、血清IgM水平及诊断情况。
共有7107例患者经sIFE确诊为单克隆丙种球蛋白病,其中377例(5.3%)为IgM型。中位年龄为62岁(范围19 - 105岁)。血清IgM的中位水平为8.3g/L(范围0.24 - 150g/L)。诊断包括意义未明的单克隆丙种球蛋白病(MGUS,157例患者,41.6%)、华氏巨球蛋白血症(WM,105例患者,27.9%)、B细胞非霍奇金淋巴瘤(69例患者,18.3%)、原发性冷凝集素病(pCAD,16例患者,4.2%)、原发性淀粉样变性(14例患者,3.7%)、冷球蛋白血症(6例患者,1.6%)、IgM MGUS相关神经病变(5例患者,1.3%)、多发性骨髓瘤(3例患者,0.8%)及POEMS综合征(2例患者,0.5%)。血清IgM>15.5g/L对WM诊断的敏感性为80.6%,特异性为89.2%。κ型轻链提示WM、pCAD、IgM MGUS相关神经病变及冷球蛋白血症的诊断,而λ型轻链提示POEMS综合征和淀粉样变性的诊断。157例MGUS患者中有41例(26.1%)被诊断为因IgM无关的自身免疫性疾病导致的并发症。
IgM单克隆丙种球蛋白病包含多种疾病。血清IgM水平及轻链类型可用于辅助鉴别诊断。MGUS与某些自身免疫性疾病之间的关联需要进一步研究。