多发性骨髓瘤轻链铸造肾病:患病率、影响和管理挑战

IF 2.1 Q2 UROLOGY & NEPHROLOGY
P. Menè, A. Stoppacciaro, S. Lai, F. Festuccia
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引用次数: 1

摘要

“铸型肾病”(Cast nephropathy, CN)是肾髓瘤的一种病理特征,在非血液学疾病引起的严重肾病综合征中也有较少的表现。由于脱水或大剂量利尿剂引起的强水重吸收引起的管腔蛋白“流”阻塞远端小管。过滤后的蛋白与内源性管状tam - horsfall糖蛋白形成复合物。在远曲小管和集管完全阻塞时,所得凝胶进一步减缓或停止管腔血流。因此,肾小管梗阻性急性肾损伤(AKI)是CN的常见后果。CN的发病机制将根据最近对B淋巴细胞单克隆疾病的理解进行综述,B淋巴细胞单克隆疾病导致免疫球蛋白成分(自由轻链,FLC)释放到血液中并通过肾小球基底膜过滤。旨在减轻FLC循环负荷的治疗,除了填补AKI发病、组织病理学诊断和药物治疗完全应答之间的空白外,还可能有助于部分患者的肾功能恢复。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Light Chain Cast Nephropathy in Multiple Myeloma: Prevalence, Impact and Management Challenges
Abstract “Cast nephropathy” (CN) is a pathological feature of myeloma kidney, also seen to a lesser extent in the context of severe nephrotic syndrome from non-haematological diseases. The name relates to obstruction of distal tubules by “casts” of luminal proteins concentrated by intensive water reabsorption resulting from dehydration or high-dose diuretics. Filtered proteins form complexes with endogenous tubular Tamm-Horsfall glycoprotein. The resulting gel further slows or stops luminal flow upon complete obstruction of distal convoluted tubules and collecting ducts. Thus, a tubular obstructive form of acute kidney injury (AKI) is a common consequence of CN. The pathogenesis of CN will be reviewed in light of recent advances in the understanding of monoclonal disorders of B lymphocytes, leading to the release of immunoglobulin components (free light chains, FLC) into the bloodstream and their filtration across the glomerular basement membrane. Treatment aiming at reduction of the circulating burden of FLC may help recovery of renal function in a fraction of these patients, besides filling the void between the onset of AKI, histopathological diagnosis, and full response to pharmacologic treatment.
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来源期刊
CiteScore
3.90
自引率
5.00%
发文量
40
审稿时长
16 weeks
期刊介绍: International Journal of Nephrology and Renovascular Disease is an international, peer-reviewed, open-access journal focusing on the pathophysiology of the kidney and vascular supply. Epidemiology, screening, diagnosis, and treatment interventions are covered as well as basic science, biochemical and immunological studies. In particular, emphasis will be given to: -Chronic kidney disease- Complications of renovascular disease- Imaging techniques- Renal hypertension- Renal cancer- Treatment including pharmacological and transplantation- Dialysis and treatment of complications of dialysis and renal disease- Quality of Life- Patient satisfaction and preference- Health economic evaluations. The journal welcomes submitted papers covering original research, basic science, clinical studies, reviews & evaluations, guidelines, expert opinion and commentary, case reports and extended reports. The main focus of the journal will be to publish research and clinical results in humans but preclinical, animal and in vitro studies will be published where they shed light on disease processes and potential new therapies and interventions.
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