Babu Adarsh, Lachmann Helen, Pickett Tom, Boddana Preetham, Ludeman Linmarie
Department of Renal Medicine, Southmead Hospital, North Bristol NHS Trust, Bristol, BS10 5NB, UK.
National Amyloidosis Centre, UCL School of Medicine, London, UK.
CEN Case Rep. 2014 May;3(1):68-74. doi: 10.1007/s13730-013-0088-8. Epub 2013 Aug 9.
A 47-year-old Caucasian man developed mild diarrhoea associated with more than 10 kg weight loss, severe fatigue and anaemia. Endoscopy demonstrated deposits of AA amyloid within the gastrointestinal tract. He had heavy proteinuria with a serum albumin of 15 g/L consistent with systemic AA amyloidosis. He had no symptoms to suggest an underlying chronic inflammatory condition but had CRP 130 mg/L and SAA 474 mg/L. In an attempt to identify the source of his inflammatory response, he underwent a contrast-enhanced whole-body computed tomography scan, which revealed a necrotising mass lesion in the right kidney consistent with a renal cell carcinoma. It also showed non-mechanical obstruction of the small bowel and, immediately post-imaging, the patient developed intractable vomiting followed by oliguric renal failure requiring haemodialysis. Despite his renal and gut failure, he underwent right radical nephrectomy without further complications. Histology showed complete resection of a clear cell renal cell carcinoma and renal amyloid deposits. Post-surgery, his acute-phase response decreased to normal, consistent with the renal cell carcinoma acting as the inflammatory stimulus. Although he remains dialysis dependent, his gut function improved and he has regained both normal weight and serum albumin. Our case demonstrates partial resolution of AA amyloidosis with removal of the inflammatory source.
一名47岁的白种男性出现轻度腹泻,伴有超过10公斤的体重减轻、严重疲劳和贫血。内镜检查显示胃肠道内有AA型淀粉样蛋白沉积。他有大量蛋白尿,血清白蛋白为15g/L,符合系统性AA型淀粉样变性。他没有提示潜在慢性炎症性疾病的症状,但CRP为130mg/L,SAA为474mg/L。为了确定其炎症反应的来源,他接受了对比增强全身计算机断层扫描,结果显示右肾有一个坏死性肿块病变,符合肾细胞癌。扫描还显示小肠存在非机械性梗阻,成像后不久,患者出现顽固性呕吐,随后发展为少尿性肾衰竭,需要进行血液透析。尽管他有肾和肠道功能衰竭,但他接受了右肾根治性切除术,未出现进一步并发症。组织学检查显示透明细胞肾细胞癌和肾淀粉样蛋白沉积均被完全切除。术后,他的急性期反应降至正常,这与肾细胞癌作为炎症刺激因素相符。尽管他仍依赖透析,但肠道功能有所改善,体重和血清白蛋白均恢复正常。我们的病例表明,去除炎症源后AA型淀粉样变性得到了部分缓解。