Mayor N, Coppola A S J, Knights Harry, Nazar T, Hunter H, Mostafid H
Stokes Centre for Urology, Royal Surrey NHS Foundation Trust, United Kingdom.
Guy's and St Thomas' NHS Foundation Trust, United Kingdom.
Int J Surg Case Rep. 2020;77:708-710. doi: 10.1016/j.ijscr.2020.11.105. Epub 2020 Nov 21.
Localised amyloidosis of the urinary tract is rare and often presents with haematuria.
A 59 year old male presented with recurrent episodes of frank haematuria exacerbated by anticoagulation after a minor stroke. He had a background of hypertension, hypercholesterolaemia, and Parkinson's disease. Initial investigations did not reveal a cause, but eventual cystoscopic biopsy showed bladder mucosa expanded by deposits of amorphous, pale, eosinophilic, proteinaceous material and immunohistochemical staining revealed the presence of amyloid deposition. Workup for systemic amyloidosis was negative. A diagnosis of primary localised amyloidosis of the bladder was made. Trans-urethral resection was performed and annual cystoscopic surveillance was commenced. He was followed up for 11 years without recurrence.
A comprehensive literature review revealed 349 published cases of localised amyloidosis of the urinary tract, with a median age of 57 (interquartile range 49-69), and a male preponderance (1.5 to 1). Painless visible haematuria (65%) was the most frequent presenting complaint and the bladder was the most common site of involvement (71%). Transurethral resection was the most common form of management (42%) but a proportion of patients underwent more radical surgery (nephroureterectomy/nephrectomy 9%, cystectomy 1%). Median follow up was 33 months (interquartile range 12-108) and 35% of patients had recurrent disease. This patient represents a typical case of localised amyloidosis of the urinary tract.
This is the most up to date review of the literature describing localised amyloidosis of the urinary tract. The disease is rare, but salient to the urologist as it invariably mimics urinary tract malignancy.
泌尿道局限性淀粉样变性罕见，常表现为血尿。
一名59岁男性，在轻度中风后因抗凝治疗而出现反复肉眼血尿发作。他有高血压、高胆固醇血症和帕金森病病史。初步检查未发现病因，但最终膀胱镜活检显示膀胱黏膜被无定形、浅色、嗜酸性、蛋白质样物质沉积物扩张，免疫组化染色显示存在淀粉样沉积。系统性淀粉样变性的检查结果为阴性。诊断为膀胱原发性局限性淀粉样变性。进行了经尿道切除术，并开始每年进行膀胱镜监测。对他进行了11年的随访，无复发。
全面的文献综述显示，有349例泌尿道局限性淀粉样变性的病例发表，中位年龄为57岁（四分位间距49 - 69岁），男性居多（1.5比1）。无痛性肉眼血尿（65%）是最常见的主诉，膀胱是最常见的受累部位（71%）。经尿道切除术是最常见的治疗方式（42%），但一部分患者接受了更激进的手术（肾输尿管切除术/肾切除术9%，膀胱切除术1%）。中位随访时间为33个月（四分位间距12 - 108个月），35%的患者有疾病复发。该患者代表了泌尿道局限性淀粉样变性的典型病例。
这是描述泌尿道局限性淀粉样变性的最新文献综述。该疾病罕见，但对泌尿外科医生很重要，因为它总是模仿泌尿道恶性肿瘤。
