肾脏单克隆γ病的意义:在三级保健中心的组织形态学谱。

Adarsh Barwad, Varun Bajaj, Geetika Singh, Amit Kumar Dinda, Ranjit Kumar Sahoo, Lalit Kumar, Sanjay Kumar Agarwal
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引用次数: 1

摘要

术语肾脏意义单克隆伽玛病(MGRS)已被描述为包括与循环单克隆蛋白相关的肾脏表现,伴或不伴克隆淋巴增生(b细胞或浆细胞),且不符合明显血液恶性肿瘤的诊断标准的患者。许多与mgrs相关的病变已经被描述为涉及不同的肾室。我们的研究描述了过去5年来我们中心的MGRS病例的组织形态学谱,并根据国际肾脏和单克隆γ病研究小组(IKMG)的分类系统进行了描述。材料与方法:回顾性分析2015 - 2020年间所有有特征性单克隆免疫球蛋白病变的肾活检进行组织病理学诊断,并由两名独立病理医师复查。结果:本组患者多处于第5个10岁,年龄中位数为50岁(平均50.14±10.43),范围为24 ~ 68岁,男性居多。多数患者以蛋白尿为唯一表现(66.6%)。多数患者(48%)血清蛋白电泳或尿蛋白电泳伴血清游离轻链异常(60.8%)出现M尖峰。AL淀粉样变是最常见的组织病理学诊断(68.7%),其次是轻链沉积病(10.4%)。结论:MGRS病变在肾脏病理学实践中很少遇到,由于一致的临床或血液学图像的限制,对诊断构成挑战。用免疫荧光和电子显微镜进行彻底的组织学检查往往有助于正确的诊断和及时的治疗。
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Monoclonal Gammopathy of Renal Significance: Histomorphological Spectrum at a Tertiary Care Center.

Introduction: 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).

Material and methods: 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.

Results: 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%).

Conclusion: 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.

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