[一例伴有血尿的患者被诊断为系统性野生型转甲状腺素淀粉样蛋白(ATTR)淀粉样变性]。

Q4 Medicine
Daichi Morooka, Yusuke Nakajima, Hidetoshi Tabata, Hiroshi Hotta
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引用次数: 0

摘要

一名 85 岁的妇女因无症状性大血尿到我院就诊。膀胱镜检查显示,该肿瘤为非乳头状无柄肿瘤,大小约 3 厘米。磁共振成像(MRI)显示肿瘤侵犯了周围的脂肪组织。胸部腹部对比增强计算机断层扫描(CT)显示上尿路无肿瘤或转移。我们诊断其为膀胱癌 cT3N0M0,并在其首次就诊 22 天后为其实施了经尿道膀胱肿瘤切除术。手术时未发现肿瘤。我们切除了一个发红的区域,包括肌肉层,并进行了随机活检。苏木精和伊红染色显示,上皮下基质和肌肉层的血管周围有嗜酸性结节组织,并染上刚果红。膀胱上皮没有发育不良。因此,我们诊断该病例为膀胱淀粉样变性。淀粉样蛋白亚型免疫染色显示为转甲状腺素淀粉样蛋白(ATTR)淀粉样变性。尿液中的本-琼斯蛋白呈阴性,血清蛋白电泳未检测到M蛋白,血清淀粉样蛋白A处于临界值。心肌99m锝焦磷酸闪烁扫描呈阳性。未发现遗传性疾病。我们得出结论,这是上述系统性 ATTRwt 淀粉样变性病。患者不愿接受系统性淀粉样变性治疗。术后 13 个月,患者的膀胱没有复发迹象。由于心脏功能是全身性淀粉样变性的预后因素,因此我们在诊断膀胱淀粉样变性时需要考虑全身性淀粉样变性的可能性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[A Case of Systemic Wild-Type Transthyretin Amyloid (ATTR) Amyloidosis Diagnosed in a Patient with Gross Hematuria].

An 85-year-old woman visited our hospital with a complaint of asymptomatic gross hematuria. Cystoscopy showed a non-papillary sessile tumor about 3 cm in size. Magnetic resonance imaging (MRI) suggested invasion of surrounding fat tissue. Thoracoabdominal contrast-enhanced computed tomography (CT) showed no tumor of the upper urinary tract or metastasis. We diagnosed the tumor as bladder cancer cT3N0M0 and performed transurethral bladder tumor resection 22 days after her first visit. No tumor was found at the time of surgery. We resected a reddened area to include a muscle layer and performed random biopsy. Hematoxylin and eosin stain showed eosinophilic tuberous tissue that stained with Congo red around blood vessels in the subepithelial stroma and the muscle layer. There was no dysplasia in the bladder epithelium. Therefore, we diagnosed the case as bladder amyloidosis. Immunostaining of the amyloid subtype revealed transthyretin amyloid (ATTR) amyloidosis. Bence-Jones protein in urine was negative, M protein was not detected in serum protein electrophoresis, and serum amyloid A was at the threshold. Scintigraphy for 99m Tc pyrophosphoric acid was positive in the myocardium. No genetic disorder was detected. We concluded that it was systemic ATTRwt amyloidosis as above. The patient did not wish to be treated for the systemic amyloidosis. Thirteen months after surgery, the patient showed no signs of recurrence in the bladder. As cardiac function is a prognostic factor in systemic amyloidosis, we need to consider the possibility of systemic amyloidosis when diagnosing bladder amyloidosis.

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来源期刊
Acta Urologica Japonica
Acta Urologica Japonica Medicine-Medicine (all)
CiteScore
0.20
自引率
0.00%
发文量
74
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