第483例——肾功能异常,间断右手麻木

The 483rd case:renal dysfunction,numbness of right hand

患者女,65岁。因血肌酐升高(155μmol/L)2月余就诊。入院后肾脏穿刺活检组织病理提示,肾小血管闭塞及肾间质纤维化,起初考虑为全身动脉粥样硬化一部分,予瑞舒伐他汀20 mg每晚1次、福辛普利10 mg每日1次、倍他乐克47.5 mg每日1次、阿司匹林0.1 g每日1次治疗,血肌酐仍波动于200μmol/L左右。进一步完善肾组织病理刚果红染色,发现砖红色物质普遍沉积于肾小叶间动脉及入球小动脉管壁内,结合血游离轻链κ340 mg/L,血游离轻链κ/λ为10.932,肾轻链型淀粉样变诊断明确。予硼替佐米(2 mg,第1、8、15、22天皮下注射)、环磷酰胺(0.3 g,第1、8、15、22天口服)、地塞米松(40 mg,第1、8、15、22天口服)方案(BCD)化疗,每28天1个疗程,化疗3个疗程后,患者达到部分缓解,血肌酐降至180μmol/L。

A 65-year-old woman presented with intermittent right hand numbness and elevated serum creatinine for more than 2 months.The histological examination of kidney biopsy showed renal arterioles occlusion and interstitial fibrosis.Pathological abnormality was originally considered as a part of systemic atherosclerosis.Thus,rosuvastatin 20 mg/d,fosinopril 10 mg/d,metoprolol 47.5 mg/d and aspirin 0.1g/d were administrated.No improvement of renal function was seen.Further Congo red staining was applied.Diffuse amorphous eosinophilic substance was deposited in interlobular artery and small arteriolar artery.Combined with the abnormal free light chain(FLC)level and ratio(serumκ340 mg/L,κ/λ10.932),the diagnosis of systematic light-chain amyloidosis was confirmed.The patient received 3 courses of chemotherapy regimen as BCD(bortezomib 2 mg d1,8,15,22,cyclophosphamide 0.3 g d1,8,15,22 and dexamethasone 40 mg d1,8,15,22).A hematologic partial response was achieved and serum creatinine decreased to 180μmol/L.