Barwad Adarsh, Bajaj Varun, Singh Geetika, Dinda Amit Kumar, Sahoo Ranjit Kumar, Kumar Lalit, Agarwal Sanjay Kumar
Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.
Department of Medical Oncology, Dr. B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India.
Glomerular Dis. 2022 Aug 4;2(4):153-163. doi: 10.1159/000526244. eCollection 2022.
The term monoclonal gammopathy of renal significance (MGRS) has been described to include patients with renal manifestations associated with circulating monoclonal proteins with or without a clonal lymphoproliferation (B-cell or plasma cell) and not meeting diagnostic criteria for an overt hematological malignancy. A host of MGRS-associated lesions have been described that involve various renal compartments. Our study describes the histomorphological spectrum of MGRS cases at our center in the last 5 years and description as per the classification system of the International Kidney and Monoclonal Gammopathy Research Group (IKMG).
Retrospective analysis was carried out of all the renal biopsies with characteristic monoclonal immunoglobulin lesions for histopathological diagnosis between years 2015 and 2020 and reviewed by two independent pathologists.
Most patients in the study belonged to the fifth decade, with a median age of 50 years (mean 50.14 ± 10.43) range (24-68 years) with a male preponderance. Most patients presented with proteinuria as the sole manifestation (66.6%). Many of the patients (48%) had an M spike by serum protein electrophoresis or urinary protein electrophoresis with an abnormal serum free light chain assay (60.8%). AL amyloidosis was the most common diagnosis observed on histopathological evaluation (68.7%), followed by light chain deposition disease (10.4%).
MGRS lesions are infrequently encountered in the practice of nephropathology and pose a diagnostic challenge due to the limitation of a congruent clinical or hematological picture. A thorough histological examination with immunofluorescence and electron microscopy often precipitates in the right diagnosis and prompts timely management.
具有肾意义的单克隆丙种球蛋白病（MGRS）这一术语已被用于描述那些伴有循环单克隆蛋白且有或无克隆性淋巴细胞增殖（B细胞或浆细胞）、但不符合明显血液系统恶性肿瘤诊断标准的有肾脏表现的患者。已经描述了许多与MGRS相关的累及肾脏不同部位的病变。我们的研究描述了过去5年我们中心MGRS病例的组织形态学谱，并按照国际肾脏与单克隆丙种球蛋白病研究组（IKMG）的分类系统进行描述。
对2015年至2020年间所有具有特征性单克隆免疫球蛋白病变的肾活检组织进行回顾性分析以进行组织病理学诊断，并由两名独立病理学家进行复查。
研究中的大多数患者为第五个十年年龄段，中位年龄为50岁（平均50.14±10.43），范围为24至68岁，男性居多。大多数患者仅表现为蛋白尿（66.6%）。许多患者（48%）通过血清蛋白电泳或尿蛋白电泳检测到M峰，且血清游离轻链检测异常（60.8%）。在组织病理学评估中观察到的最常见诊断是AL淀粉样变性（68.7%），其次是轻链沉积病（10.4%）。
MGRS病变在肾病病理学实践中并不常见，由于缺乏一致的临床或血液学表现，构成了诊断挑战。通过免疫荧光和电子显微镜进行全面的组织学检查通常有助于做出正确诊断并促使及时治疗。
