揭开肾脏单克隆丙种球蛋白病的神秘面纱:关于肾脏并发症和临床见解的微型综述。

Frontiers in nephrology Pub Date : 2024-09-12 eCollection Date: 2024-01-01 DOI:10.3389/fneph.2024.1439288
Mythri Shankar, Manjusha Yadla
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引用次数: 0

摘要

肾脏重要单克隆抗体病(MGRS)是指由于异常单克隆蛋白的积累或影响而导致的肾脏损伤。这些蛋白质来源于非癌或癌前浆细胞或 B 细胞,沉积在肾脏的特定区域。导致 MGRS 的机制包括血管内皮生长因子的高水平分泌、针对补体成分的自身抗体以及针对特定受体导致的肾病。肾脏单克隆丙种球蛋白病(MGRS)的肾脏病变可根据有组织或无组织沉积物(包括纤维状、微管状或晶体包涵体)的存在进行分类。肾活检是通过鉴别单克隆免疫球蛋白沉积物确诊 MGRS 的关键。免疫荧光有助于确定 MGRS 所涉及的轻链和/或重链类别。治疗方法以克隆为导向,因此取决于是否存在 B 细胞克隆或浆细胞克隆或任何可检测到的单克隆蛋白。针对浆细胞或 B 细胞恶性肿瘤的化疗和自体造血细胞移植可用于治疗 MGRS。MGRS患者的肾脏预后与化疗的血液学反应密切相关。
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Unraveling monoclonal gammopathy of renal significance: a mini review on kidney complications and clinical insights.

Monoclonal gammopathy of renal significance (MGRS) is where kidney injury occurs due to the accumulation or effects of abnormal monoclonal proteins. These proteins, originating from non-cancerous or pre-cancerous plasma cells or B cells, deposit in specific areas of the kidney. Mechanisms contributing to MGRS include high levels of vascular endothelial growth factor secretion, autoantibodies targeting complement components, and targeting specific receptors leading to nephropathy. Kidney lesions in monoclonal gammopathy of renal significance (MGRS) are classified based on the presence of organized or nonorganized deposits, including fibrillar, microtubular, or crystal inclusions. Kidney biopsy is essential for confirming the diagnosis of MGRS by identifying monoclonal immunoglobulin deposits. Immunofluorescence helps determine the class of light and/or heavy chain involved in MGRS. The treatment approach is clone-directed and hence it depends on the presence of B cell clone or plasma cell clone or any detectable monoclonal protein. Chemotherapy targeting plasma cell or B cell malignancies and autologous hematopoietic cell transplantation may be used to manage MGRS. Kidney outcomes in MGRS patients strongly correlate with the hematologic response to chemotherapy.

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