Fine J M, Marneux M
Rev Fr Transfus Immunohematol. 1985 Dec;28(6):591-600. doi: 10.1016/s0338-4535(85)80004-0.
Monoclonal gammopathies (M.G.) are a group of disorders characterized by the proliferation of a single clone of plasma-cells that produce a Monoclonal Immunoglobulin (M-Ig). The presence of M-Ig can be demonstrated by electrophoresis in the patient' serum and identified by immunoelectrophoresis as a molecule containing two heavy chains of a single class and two light chains of a single type. In M.G. of malignant origin, free light chains (Bence Jones Proteins) can be evidenced in the concentrated urines. The fact that M.G. may be more frequent in certain families show the existence of a familial predisposition of this disease whereas its origin is still unknown. M.G. are often associated with malignant proliferation of B lymphocytes such as multiple myeloma, Waldenström's macroglobulinemia, heavy chain disease as well as some other lymphoproliferative disorders. However, in a certain number of cases, the malignant origin of the M.G. was not proved, because M-Ig can occur in the serum of people apparently in good health and without clinical or hematological features (asymptomatic "benign" M.G.). Asymptomatic benign M.G. have been detected in increasing numbers during the last decade due to use of cellulose acetate electrophoresis for the routine examination of patients or in the course of systemic screening in normal populations such as blood donors. At the present time, a malignant origin of M.G. cannot be proved in more than thirty per cent of case and these "asymptomatic M.G." must be follow-up by a yearly clinical, hematological and electrophoretical check-up in order to detect a possible malignant evolution. In other cases, M.G. can be associated with neoplasms of cells types not known to produce M-Ig, in cold chronic agglutinin disease, and during the course of some auto-immune disorders.
单克隆丙种球蛋白病(M.G.)是一组以单个浆细胞克隆增殖并产生单克隆免疫球蛋白(M-Ig)为特征的疾病。M-Ig的存在可通过患者血清电泳证实,并通过免疫电泳鉴定为一种含有单一类别的两条重链和单一类型的两条轻链的分子。在恶性起源的M.G.中,浓缩尿中可检测到游离轻链(本周蛋白)。M.G.在某些家族中可能更常见,这一事实表明该疾病存在家族易感性,但其病因仍不清楚。M.G.常与B淋巴细胞的恶性增殖有关,如多发性骨髓瘤、华氏巨球蛋白血症、重链病以及其他一些淋巴增殖性疾病。然而,在一定数量的病例中,M.G.的恶性起源未得到证实,因为M-Ig可出现在健康状况良好且无临床或血液学特征的人的血清中(无症状的“良性”M.G.)。由于在患者常规检查中使用醋酸纤维素电泳或在正常人群(如献血者)的系统筛查过程中,无症状良性M.G.在过去十年中被检测到的数量不断增加。目前,超过30%的病例无法证实M.G.的恶性起源,这些“无症状M.G.”必须每年进行临床、血液学和电泳检查以监测是否可能发生恶性演变。在其他情况下,M.G.可与未知产生M-Ig的细胞类型的肿瘤相关,如冷凝集素病以及某些自身免疫性疾病过程中。