Grigor Thomas, Munro Nicolas
Department of Histopathology, Royal Cornwall Hospital, Truro, UK.
Royal Cornwall Hospital, Truro, UK.
BMJ Case Rep. 2015 Jan 16;2015:bcr2014207955. doi: 10.1136/bcr-2014-207955.
We describe a rare case of light chain immunoglobulin amyloid (AL) accumulation in the central and lower pole renal calyces. Our patient, a woman aged 60, presented with several episodes of gross haematuria. Radiological imaging detected a filling defect in the left renal pelvis. Rigid ureteroscopy showed a corresponding mucosal abnormality resembling transitional cell carcinoma. A definitive preoperative tissue diagnosis could not be reached. Laparoscopic-assisted left nephroureterectomy was indicated. Histopathological examination excluded malignancy, revealing congophilic deposits of submucosal amyloid. A constellation of findings confirmed localised or primary amyloidosis with an AL immunophenotype but no evidence of clonal B-cell disease in the amyloid-associated lymphoplasmacytic cell infiltrate. Investigation for systemic plasma cell dyscrasia and echocardiography and scintigraphy for visceral amyloid deposits were negative for systemic disease. At a follow-up period of 30 months, there is no recurrence. However, our patient was diagnosed with breast cancer 21 months ago.
我们描述了一例罕见的轻链免疫球蛋白淀粉样变性(AL)在肾盂中央和下极积聚的病例。我们的患者为一名60岁女性,出现了几次肉眼血尿。影像学检查发现左肾盂有充盈缺损。硬性输尿管镜检查显示相应的黏膜异常,类似移行细胞癌。术前未能获得明确的组织诊断。遂行腹腔镜辅助下左肾输尿管切除术。组织病理学检查排除了恶性肿瘤,显示黏膜下淀粉样物质呈嗜刚果红染色沉积。一系列检查结果证实为具有AL免疫表型的局限性或原发性淀粉样变性,但在淀粉样物质相关的淋巴浆细胞浸润中未发现克隆性B细胞疾病的证据。对系统性浆细胞异常增生的检查以及对内脏淀粉样物质沉积的超声心动图和闪烁扫描检查均未发现系统性疾病。在30个月的随访期内,无复发。然而,我们的患者在21个月前被诊断出患有乳腺癌。