由电子发光晶体引起的轻链结晶足细胞病——一个诊断挑战:1例报告。

IF 1.1 4区 医学 Q4 MICROSCOPY
Rajib K Gupta, Maria E Pagtalunan, Ann M Wexler, Joseph M Tuscano
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引用次数: 0

摘要

我们在此报告一个具有挑战性的诊断为轻链结晶足细胞病的患者,该患者患有亚肾病性蛋白尿,显微镜下血尿,血糖升高和游离kappa轻链升高,但没有任何明确的多发性骨髓瘤的初步临床证据。我们还讨论了该病例的诊断挑战,包括肾小球的相当独特和看似无害的光镜形态和石蜡免疫荧光的关键作用,以及需要电子显微镜来确认诊断。仅用石蜡免疫荧光法证实了足细胞内晶体的kappa限制(常规免疫荧光法未检测到),肾小球的电镜显示足细胞病变是由电子透明晶体引起的,这是一种罕见的轻链晶体变体,电子致密晶体是轻链晶体的主要变体。
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 crystalline podocytopathy caused by electron-lucent crystals - a diagnostic challenge: a case report.

We report here a challenging diagnosis of light chain crystalline podocytopathy in a patient with sub-nephrotic proteinuria, microscopic hematuria, glucosuria and elevated free kappa light chains but without any initial clinical evidence of frank multiple myeloma. We also discuss the diagnostic challenges of this case which include rather unique and seemingly innocuous light microscopic morphology of the glomeruli and the critical roles of paraffin immunofluorescence and need for electron microscopy to confirm the diagnosis. The kappa restriction of the crystals within the podocytes was confirmed only by paraffin immunofluorescence (it went undetected by routine immunofluorescence) and electron microscopy of the glomeruli showed the podocytopathy to be caused by electron-lucent crystals which is a rare variant of light chain crystals, with electron-dense crystals being the predominant variant of light chain crystals.

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来源期刊
Ultrastructural Pathology
Ultrastructural Pathology 医学-病理学
CiteScore
2.00
自引率
10.00%
发文量
40
审稿时长
6-12 weeks
期刊介绍: Ultrastructural Pathology is the official journal of the Society for Ultrastructural Pathology. Published bimonthly, we are the only journal to be devoted entirely to diagnostic ultrastructural pathology. Ultrastructural Pathology is the ideal journal to publish high-quality research on the following topics: Advances in the uses of electron microscopic and immunohistochemical techniques Correlations of ultrastructural data with light microscopy, histochemistry, immunohistochemistry, biochemistry, cell and tissue culturing, and electron probe analysis Important new, investigative, clinical, and diagnostic EM methods.
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