Shibasaki T, Matsumoto H, Watabe K, Joh K, Nakano H, Matsuda H, Gomi H, Ohno I, Ishimoto F, Sakai O
Department of Internal Medicine (II), The Jikei University School of Medicine, Minato-ku, Tokyo, Japan.
Nephron. 1997;75(3):350-3. doi: 10.1159/000189560.
We report a case of a 35-year-old man with secondary amyloidosis chiefly involving the kidney and heart. The patient showed severe proteinuria and ischemic heart damage and had hepatic adenoma at the age of 33. Biopsy specimens from the kidney, heart, stomach and rectum showed extensive deposition of amyloid. After the surgical resection of a 300-gram hepatic adenoma, highly elevated c-reactive protein (CRP) levels decreased and the serum amyloid A (SAA) level was completely normalized. Normal liver cells were immunostained with rabbit anti-SAA antibody, but the cells in adenoma tissue and kidney were not. Electron microscopic examination revealed extracellular deposition of amyloid fibrils in the hepatic adenoma and kidney tissue. The concentration of tumor necrosis factor-alpha (TNF-alpha) (312 pg/mg tissue protein) was 7-fold higher in adenoma tissue than in normal liver tissue. Moreover, SAA (2.8 ng/mg tissue protein) was 2-fold higher in normal liver tissue than in adenoma tissue. Since TNF-alpha has been known to induce SAA production in target cells, the present results suggest that the hepatic adenoma produced TNF-alpha, which then caused mainly secondary amyloidosis in the kidney and heart. Currently, 2 years after surgical resection, urinary excretion of protein has been markedly reduced (from 3.5 to 0.8 g/day) and renal and cardiac functions are normal without specific medical treatment.
我们报告一例35岁男性继发性淀粉样变性病例，主要累及肾脏和心脏。该患者出现严重蛋白尿和缺血性心脏损害，33岁时患有肝腺瘤。肾脏、心脏、胃和直肠的活检标本显示有广泛的淀粉样物质沉积。在手术切除一个300克的肝腺瘤后，高度升高的C反应蛋白（CRP）水平下降，血清淀粉样蛋白A（SAA）水平完全恢复正常。正常肝细胞用兔抗SAA抗体进行免疫染色，但腺瘤组织和肾脏中的细胞未被染色。电子显微镜检查显示肝腺瘤和肾脏组织中有淀粉样原纤维的细胞外沉积。腺瘤组织中肿瘤坏死因子-α（TNF-α）（312 pg/mg组织蛋白）的浓度比正常肝组织高7倍。此外，正常肝组织中的SAA（2.8 ng/mg组织蛋白）比腺瘤组织高2倍。由于已知TNF-α可诱导靶细胞产生SAA，目前的结果表明肝腺瘤产生了TNF-α，进而主要导致了肾脏和心脏的继发性淀粉样变性。目前，手术切除后2年，尿蛋白排泄量已显著减少（从3.5克/天降至0.8克/天），肾脏和心脏功能正常，无需特殊药物治疗。
