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伴有尿红细胞管型的非典型AL淀粉样变性病例。

Atypical case of AL amyloidosis with urinary erythrocyte casts.

作者信息

Liangos Orfeas, Buettner-Herold Maike, Ketteler Markus, Madias Nicolaos E

机构信息

Klinikum Coburg, III. Medizinische Klinik, Coburg.

Department of Anatomic Pathology, Section on Nephropathology, University of Erlangen Nuremberg, Erlangen, Germany, and.

出版信息

Clin Nephrol Case Stud. 2015 Sep 23;3:19-24. doi: 10.5414/CNCS108640. eCollection 2015.

Abstract

We present the case of a 73-year-old man who developed an acute, severe febrile illness with multiorgan dysfunction, featuring renal failure, nephrotic-range proteinuria, microhematuria, and a skin rash. Numerous erythrocyte casts were found on urine microscopy. Typically, the finding of urinary erythrocyte casts indicates the presence of an underlying glomerular inflammatory disease. However, on renal biopsy, only amyloid light-chain (AL) amyloidosis and tubular injury were the predominant findings with no signs of glomerular or vascular inflammation. Photomicrographs of urinary sediment as well as renal biopsy histopathology of the presented case are shown. The unusual combination of findings, is then discussed in light of the existing literature on renal amyloidosis as well as erythrocyte casts in conditions other than glomerulonephritis.

摘要

我们报告了一例73岁男性患者,该患者出现了一种急性、严重的发热性疾病,并伴有多器官功能障碍,其特征为肾衰竭、肾病范围蛋白尿、镜下血尿和皮疹。尿液显微镜检查发现大量红细胞管型。通常,尿液中发现红细胞管型表明存在潜在的肾小球炎症性疾病。然而,肾活检显示,主要发现仅为淀粉样轻链(AL)淀粉样变性和肾小管损伤,未发现肾小球或血管炎症的迹象。文中展示了该病例的尿沉渣显微照片以及肾活检组织病理学图像。然后,结合关于肾淀粉样变性以及除肾小球肾炎外其他病症中红细胞管型的现有文献,对这些不寻常的发现组合进行了讨论。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/09d1/5438007/8f1cbba2fdb4/CNCS-3-019-01.jpg

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