Netti Giuseppe Stefano, Troise Dario, Rossini Michele, Catalano Valeria, De Luca Federica, Khalid Javeria, Camporeale Valentina, Ritrovato Fabiana, Infante Barbara, Sanguedolce Francesca, Stallone Giovanni, Ranieri Elena
Unit of Clinical Pathology, Department of Medical and Surgical Sciences, University of Foggia, University Hospital "Policlinico Riuniti", Viale Luigi Pinto, 71122 Foggia, Italy.
Center for Research and Innovation in Medicine (CREATE), Department of Medical and Surgical Sciences, University of Foggia, University Hospital "Policlinico Riuniti", Viale Luigi Pinto, 71122 Foggia, Italy.
Diagnostics (Basel). 2024 Dec 23;14(24):2892. doi: 10.3390/diagnostics14242892.
Monoclonal gammopathy of renal significance (MGRS) refers to a group of renal disorders caused by a monoclonal immunoglobulin (MIg), secreted by a non-malignant B-cell clone. Unlike overt multiple myeloma or B-cell proliferation, MGRS does not meet those diagnostic criteria. However, it is associated with significant morbidity, due to severe renal, and sometimes systemic, lesions induced by the MIg. Early recognition is crucial, as chemotherapy to suppress MIg secretion often improves outcomes. The spectrum of renal diseases in MGRS is broad, including both well-known conditions like AL amyloidosis and newly described lesions. Kidney biopsy is essential to determine the specific lesion associated with MGRS and assess its severity. Diagnosis involves integrating morphologic alterations using techniques such as light microscopy, immunofluorescence (IF), electron microscopy, and, in some cases, IF staining for Ig isotypes, immunoelectron microscopy, and proteomic analysis. Additionally, a complete hematologic evaluation, including serum and urine protein electrophoresis, immunofixation, and a serum-free light-chain assay, is necessary.
具有肾意义的单克隆丙种球蛋白病(MGRS)指的是一组由非恶性B细胞克隆分泌的单克隆免疫球蛋白(MIg)引起的肾脏疾病。与明显的多发性骨髓瘤或B细胞增殖不同,MGRS不符合那些诊断标准。然而,由于MIg引起的严重肾脏病变,有时还包括全身性病变,它与显著的发病率相关。早期识别至关重要,因为抑制MIg分泌的化疗通常会改善预后。MGRS中的肾脏疾病谱很广,包括如AL淀粉样变性等知名病症和新描述的病变。肾活检对于确定与MGRS相关的特定病变并评估其严重程度至关重要。诊断需要整合形态学改变,使用诸如光学显微镜、免疫荧光(IF)、电子显微镜等技术,在某些情况下还需要进行Ig同种型的IF染色、免疫电子显微镜和蛋白质组分析。此外,还需要进行完整的血液学评估,包括血清和尿蛋白电泳、免疫固定和血清游离轻链检测。