Gupta Vinay, El Ters Mireille, Kashani Kianoush, Leung Nelson, Nasr Samih H
Divisions of Hematology.
Nephrology and Hypertension.
J Am Soc Nephrol. 2015 Mar;26(3):525-9. doi: 10.1681/ASN.2014050509. Epub 2014 Sep 4.
Crystalline nephropathy refers to renal parenchymal deposition of crystals leading to kidney damage. The most common forms of crystalline nephropathy encountered in renal pathology are nephrocalcinosis and oxalate nephropathy. Less frequent types include urate nephropathy, cystinosis, dihydroxyadeninuria, and drug-induced crystalline nephropathy (e.g., caused by indinavir or triamterene). Monoclonal proteins can also deposit in the kidney as crystals and cause tissue damage. This occurs in conditions such as light chain proximal tubulopathy, crystal-storing histiocytosis, and crystalglobulinemia. The latter is a rare complication of multiple myeloma that results from crystallization of monoclonal proteins in the systemic vasculature, leading to vascular injury, thrombosis, and occlusion. In this report, we describe a case of crystalglobulin-induced nephropathy and discuss its pathophysiology and the differential diagnosis of paraprotein-induced crystalline nephropathy.
结晶性肾病是指晶体在肾实质沉积导致肾损伤。肾病理学中最常见的结晶性肾病形式是肾钙质沉着症和草酸盐肾病。较少见的类型包括尿酸盐肾病、胱氨酸病、二羟腺嘌呤尿症和药物性结晶性肾病(如由茚地那韦或氨苯蝶啶引起)。单克隆蛋白也可作为晶体沉积在肾脏并导致组织损伤。这发生在轻链近端肾小管病、晶体贮积性组织细胞增多症和晶体球蛋白血症等情况中。后者是多发性骨髓瘤的一种罕见并发症,由单克隆蛋白在全身血管系统中结晶所致,导致血管损伤、血栓形成和阻塞。在本报告中,我们描述了一例晶体球蛋白诱导的肾病病例,并讨论其病理生理学及副蛋白诱导的结晶性肾病的鉴别诊断。