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一例肾和脾 LECT 2 淀粉样变性:一种新近被认识的肾和系统性淀粉样变性病因。

A case of renal and splenic LECT 2 amyloidosis: A recently recognized cause of renal and systemic amyloidosis.

机构信息

Department of Medicine, Division of Nephrology, UCLA David Geffen School of Medicine, Los Angeles, CA, USA.

Department of Pathology, Division of Renal Pathology, UCLA David Geffen School of Medicine, Los Angeles, CA, USA.

出版信息

Saudi J Kidney Dis Transpl. 2020 Mar-Apr;31(2):508-514. doi: 10.4103/1319-2442.284027.

Abstract

Amyloidosis has traditionally been of a few defined varieties, most commonly including light-chain amyloidosis (AL amyloidosis) and secondary amyloidosis due to chronic inflammation (AA amyloidosis). Apolipoprotein A-I/A-II cystatin C, gelsolin, lysozyme, fibrinogen alpha chain, beta 2 microglobulin, and transthyretin familial amyloidosis represent rarer but reported varieties. Ten years ago, the first reports linked leukocyte chemotactic factor 2 (LECT2) amyloidosis as a pathological agent identified as a novel class of amyloid-generating protein. Epidemiology suggested that this was a new cause of amyloidosis that is especially common in Hispanic patients and somewhat common among patients from the Middle East-North Africa (MENA) region. We report a case of splenic and renal LECT 2 amyloidosis in a 62-year- old Hispanic male with diabetes mellitus. After an unremarkable serological workup, LECT 2 amyloidosis was diagnosed on renal biopsy. The case presentation is reviewed as a typical presentation, and the literature is reviewed regarding this newly reported entity, resulting in infiltrative renal amyloidosis and chronic renal disease.

摘要

淀粉样变性传统上有几种明确的类型,最常见的包括轻链淀粉样变性(AL 淀粉样变性)和继发于慢性炎症的淀粉样变性(AA 淀粉样变性)。载脂蛋白 A-I/A-II 胱抑素 C、胶凝蛋白、溶菌酶、纤维蛋白原α链、β2 微球蛋白和转甲状腺素家族性淀粉样变性是更为罕见但有报道的类型。十年前,首次有报道将白细胞趋化因子 2(LECT2)淀粉样变性作为一种病理物质,被确定为一类新的产生淀粉样物质的蛋白质。流行病学表明,这是一种新的淀粉样变性病因,尤其常见于西班牙裔患者,在中东-北非(MENA)地区的患者中也有些常见。我们报告了一例 62 岁西班牙裔男性糖尿病患者的脾脏和肾脏 LECT 2 淀粉样变性。在进行了无明显异常的血清学检查后,在肾活检中诊断出 LECT 2 淀粉样变性。本文回顾了该病例的临床表现,是一个典型的表现,并对该新报告实体的文献进行了回顾,导致浸润性肾淀粉样变性和慢性肾病。

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