Javaugue V, Bouteau I, Sirac C, Quellard N, Diolez J, Colombo A, Desport E, Ecotière L, Goujon J-M, Fermand J-P, Touchard G, Jaccard A, Bridoux F
Service de néphrologie, hémodialyse et transplantation rénale, centre national de référence maladies rares : amylose AL et autres maladies par dépôts d'immunoglobulines monoclonales, CHU de Poitiers, 2, rue de la Milétrie, 86021 Poitiers, France; CNRS-UMR 7276, centre national de référence maladies rares : amylose AL et autres maladies par dépôts d'immunoglobulines monoclonales, université de Limoges, 87000 Limoges, France.
Service de néphrologie, hémodialyse et transplantation rénale, centre national de référence maladies rares : amylose AL et autres maladies par dépôts d'immunoglobulines monoclonales, CHU de Poitiers, 2, rue de la Milétrie, 86021 Poitiers, France.
Rev Med Interne. 2018 Mar;39(3):161-170. doi: 10.1016/j.revmed.2017.03.012. Epub 2017 Apr 27.
Two categories of renal disorders associated with monoclonal gammopathies are to be distinguished, according to the characteristics of the underlying B-cell clone. The first group of renal diseases always occurs in the setting of high tumor mass with production of large amounts of monoclonal immunoglobulins. The main complication is the so-called myeloma cast nephropathy, which almost invariably complicates high tumor mass myeloma. The second group includes all renal disorders caused by a monoclonal immunoglobulin secreted by a nonmalignant B-cell clone, and currently referred as a "monoclonal gammopathy of renal significance (MGRS)". This term was introduced to distinguish monoclonal gammopathies that are responsible for the development of kidney damage from those that are truly benign. The spectrum of renal diseases in MGRS is wide and its classification relies on the localization of renal lesions, either glomerular or tubular, and on the pattern of ultrastructural organization of immunoglobulin deposits. Physicochemical characteristics of the pathogenic monoclonal immunoglobulin are probably involved in their propensity to deposit or precipitate in the kidney, as illustrated by the high rate of recurrence of each specific type after kidney transplantation. Early diagnosis and efficient chemotherapy targeting the causal B-cell clone are mandatory to improve renal prognosis and patient survival.
根据潜在B细胞克隆的特征,可区分出两类与单克隆丙种球蛋白病相关的肾脏疾病。第一组肾脏疾病总是发生在肿瘤负荷高且产生大量单克隆免疫球蛋白的情况下。主要并发症是所谓的骨髓瘤管型肾病,几乎总是使高肿瘤负荷的骨髓瘤复杂化。第二组包括由非恶性B细胞克隆分泌的单克隆免疫球蛋白引起的所有肾脏疾病,目前称为“具有肾脏意义的单克隆丙种球蛋白病(MGRS)”。引入这个术语是为了区分导致肾损害的单克隆丙种球蛋白病和真正良性的单克隆丙种球蛋白病。MGRS中的肾脏疾病谱很广,其分类依赖于肾脏病变的定位,无论是肾小球还是肾小管,以及免疫球蛋白沉积物的超微结构组织模式。致病性单克隆免疫球蛋白的物理化学特性可能与其在肾脏中沉积或沉淀的倾向有关,肾脏移植后每种特定类型的高复发率就说明了这一点。早期诊断和针对病因性B细胞克隆的有效化疗对于改善肾脏预后和患者生存率至关重要。