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Primary Localized Amyloidosis of Urinary Bladder: A Case Report and Review of Literature

机译:原发性膀胱局部淀粉样变性病:一例报道并文献复习

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摘要

Amyloidosis is a disorder of protein metabolism characterized by extracellular deposition of abnormal protein fibrils. It may either be localized to any organ or systematically distributed throughout the body. The biochemical nature of proteins varies but the physical and tinctorial properties are shared by all the amyloidogenic proteins. In the West, it is mainly composed of amyloid light (AL) type immunoglobulin (Ig) light chains. Amyloidosis of the genitourinary tract is rare except for the kidney and isolated primary amyloidosis of the urinary bladder is even rarer. It mainly presents as intermittent painless gross hematuria. It mimics transitional cell carcinoma on imaging and endoscopic examination. We herein present a case of fifty six-years-old male with history of painless hematuria for three months. Cystoscopy revealed a 1 cm hyperemic area on the posterior wall of urinary bladder. The biopsy showed features of amyloidosis and amyloid A (AA) immunostaining was negative. Extensive workup was done to exclude other sites of involvement and a final diagnosis of primary localized amyloidosis of the urinary bladder was made. The patient is on regular follow-up.
机译:淀粉样变性是一种蛋白质代谢异常,其特征在于异常蛋白质原纤维的细胞外沉积。它可以定位于任何器官,也可以系统地分布在全身。蛋白质的生化性质各不相同,但所有淀粉样蛋白均具有物理和着色特性。在西方,它主要由淀粉样蛋白轻(AL)型免疫球蛋白(Ig)轻链组成。除肾脏外,泌尿生殖道的淀粉样变性很少见,而孤立的原发性膀胱淀粉样变性则更为罕见。它主要表现为间歇性无痛性肉眼血尿。它在成像和内窥镜检查中模仿移行细胞癌。我们在此介绍一例五十岁的男性,有无痛性血尿病史三个月。膀胱镜检查发现膀胱后壁有1 cm充血区。活检显示淀粉样变性病的特征,淀粉样蛋白A(AA)免疫染色为阴性。进行了广泛的检查以排除其他受累部位,并最终诊断出原发性膀胱局限性淀粉样变性。患者正在定期随访中。

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