Wu Xin-Yao, Yin Yu-Feng, Teng Jia-Lin, Zhang Li-Wei, Yang Cheng-de
Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, No. 197 Ruijin Second Road, Huangpu District, Shanghai, 200025, China.
Department of Clinical Laboratory, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, 200025, China.
BMC Immunol. 2017 Jun 23;18(1):32. doi: 10.1186/s12865-017-0213-0.
The monoclonal gammopathies are a group of plasma-cell proliferative disorders characterized by the secretion of monoclonal immunoglobulin (M protein or paraprotein). Some rare cases have revealed the specific affinity of paraprotein as autoantibody. Here we report a patient with monoclonal gammopathy of undetermined significance (MGUS) accompanied by a remarkable increase of anticardiolipin antibody (aCL) and an extensively decreased coagulation factor activity, however, without any clinical signs of antiphospholipid syndrome (APS) and bleeding.
Our further investigation indicated that IgMκ paraprotein of this patient possessed an antibody activity against phospholipids so as to bind to cardiolipin and interfere with coagulation assay in vitro.
This case might be indicative that an abnormality of coagulation tests, disturbed by IgMκ paraprotein, does not predict a risk of bleeding in this patient.
单克隆丙种球蛋白病是一组浆细胞增殖性疾病,其特征是分泌单克隆免疫球蛋白(M蛋白或副蛋白)。一些罕见病例显示副蛋白具有自身抗体的特异性亲和力。在此,我们报告一例意义未明的单克隆丙种球蛋白病(MGUS)患者,其抗心磷脂抗体(aCL)显著增加,凝血因子活性广泛降低,但无任何抗磷脂综合征(APS)和出血的临床症状。
我们的进一步研究表明,该患者的IgMκ副蛋白具有抗磷脂抗体活性,从而在体外与心磷脂结合并干扰凝血检测。
该病例可能表明,IgMκ副蛋白干扰的凝血试验异常并不能预测该患者的出血风险。