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横纹肌溶解症和急性肾功能障碍作为具有肾意义的单克隆丙种球蛋白病的初始表现

Rhabdomyolysis and Acute Renal Dysfunction as Initial Manifestations of Monoclonal Gammopathy of Renal Significance.

作者信息

Gudapati Prathyusha, Al-Sultani Anmar, Parmar Ashish, Motwani Reena, Fortkort Peter

机构信息

Internal Medicine, UNC Health Southeastern, Lumberton, USA.

Internal Medicine, Campbell University School of Osteopathic Medicine, Buis Creek, USA.

出版信息

Cureus. 2023 Feb 8;15(2):e34759. doi: 10.7759/cureus.34759. eCollection 2023 Feb.

Abstract

Monoclonal gammopathy of renal significance (MGRS) is a rare heterogeneous group of kidney disorders that encompasses all disorders caused by deposition of monoclonal protein (M-protein) and its light or heavy chain fragments secreted by pre-malignant or non-malignant B-cell clones in patients who do not meet the diagnostic criteria for multiple myeloma (MM) or other B-cell malignancies. MGRS can manifest as glomerular diseases, tubulopathies, or vascular involvement with varying clinical presentations, making the diagnosis of MGRS challenging. In patients with high clinical suspicion based on preliminary blood and urine studies, the evaluation of MGRS begins with a renal biopsy followed by monoclonal studies and cytogenetic analysis. There is no standard treatment protocol for MGRS, and the current consensus suggests a clone-directed approach. If not identified and treated early, MGRS often results in poor outcomes and can lead to extrarenal manifestations, such as cardiogenic shock. Herein, we present a case involving a 43-year-old male with a rare presentation of rhabdomyolysis, rapidly progressing renal dysfunction, and cardiac dysfunction. A bone marrow biopsy did not meet the diagnostic criteria for MM or other B-cell malignancies, while a renal biopsy revealed Kappa light chain cast nephropathy, which led to the final diagnosis of MGRS.

摘要

具有肾脏意义的单克隆丙种球蛋白病(MGRS)是一组罕见的异质性肾脏疾病,涵盖了所有由单克隆蛋白(M蛋白)及其轻链或重链片段沉积引起的疾病,这些片段由不符合多发性骨髓瘤(MM)或其他B细胞恶性肿瘤诊断标准的患者的癌前或非恶性B细胞克隆分泌。MGRS可表现为肾小球疾病、肾小管病变或血管受累,临床表现各异,这使得MGRS的诊断具有挑战性。对于基于初步血液和尿液检查高度怀疑MGRS的患者,其评估始于肾活检,随后进行单克隆研究和细胞遗传学分析。MGRS没有标准的治疗方案,目前的共识是采用针对克隆的方法。如果不及早识别和治疗,MGRS通常会导致不良后果,并可能导致肾外表现,如心源性休克。在此,我们报告一例43岁男性病例,其表现为罕见的横纹肌溶解、快速进展的肾功能不全和心功能不全。骨髓活检不符合MM或其他B细胞恶性肿瘤的诊断标准,而肾活检显示κ轻链管型肾病,最终诊断为MGRS。

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