CAJ | 학술논문

目的总结肾脏原发系统性轻链型淀粉样变患者的临床病理特征,探讨影响预后的因素.方法回顾性分析25例肾脏原发系统性轻链型淀粉样变患者的临床资料,随访其生存情况.结果 25例肾脏原发系统性轻链型淀粉样变患者,中位年龄57(37～69)岁,游离λ轻链型占绝大多数(88%,22/25).突出症状为大量蛋白尿、肾病综合征伴顽固性水肿,尿蛋白排泄以白蛋白为主.肾脏病理病变主要累及肾小球系膜区局灶性或弥漫性无细胞性明显增宽.25例患者诊断后中位生存时间24.4个月,1、2和3年总生存率分别为(65±10)%、(46±12)%和(15±12)%.明确诊断时单独肾脏淀粉样变14例,淀粉样变同时累及其他脏器组织11例,两者诊断后中位生存时间分别为24.7个月和16.4个月,差异有统计学意义(P=0.03).Cox回归单因素分析表明:年龄、性别、骨髑浆细胞比例、白蛋白和血红蛋白水平与预后无关(P＞0.05),肾功能损害和淀粉样变同时累及其他脏器与预后相关(P＜0.05),其中同时有心脏病变很可能与预后呈负相关(P=0.06),进一步Cox逐步回归多因素分析表明肾功能损害是影响肾脏原发系统性轻链型淀粉样变患者预后的一个独立危险因素(P＜0.05).结论肾功能损害是影响预后的独立高危因素,同时联合有其他脏器尤其是心脏淀粉样变患者预后差.
목적 총결신장원발계통성경련형정분양변환자적림상병리특정,탐토영향예후적인소.방법 회고성분석25례신장원발계통성경련형정분양변환자적림상자료,수방기생존정황.결과 25례신장원발계통성경련형정분양변환자,중위년령57(37～69)세,유리λ경련형점절대다수(88%,22/25).돌출증상위대량단백뇨、신병종합정반완고성수종,뇨단백배설이백단백위주.신장병리병변주요루급신소구계막구국조성혹미만성무세포성명현증관.25례환자진단후중위생존시간24.4개월,1、2화3년총생존솔분별위(65±10)%、(46±12)%화(15±12)%.명학진단시단독신장정분양변14례,정분양변동시루급기타장기조직11례,량자진단후중위생존시간분별위24.7개월화16.4개월,차이유통계학의의(P=0.03).Cox회귀단인소분석표명:년령、성별、골촉장세포비례、백단백화혈홍단백수평여예후무관(P＞0.05),신공능손해화정분양변동시루급기타장기여예후상관(P＜0.05),기중동시유심장병변흔가능여예후정부상관(P=0.06),진일보Cox축보회귀다인소분석표명신공능손해시영향신장원발계통성경련형정분양변환자예후적일개독립위험인소(P＜0.05).결론 신공능손해시영향예후적독립고위인소,동시연합유기타장기우기시심장정분양변환자예후차.
Objective To analyze the clinical pathology features of light-chain amyloidosis associated renal disease,and investigate the survival influential factors. Method From January 1998 to March 2009,25 patients with light-chain amyloidosis associated renal disease were reviewed and followed up.Results Of the 25 patients with light-chain amyloidosis associated renal disease,median age was 57(37-69) years old and lamda light-chain predominated (88% ,22/25). Heavy proteinuria and nephrotic syndrome with peripheral edema were typical clinical presentations. Renal biopsy showed that amyloid deposition of light-chain amyloidosis associated renal disease involved the glomeruh mostly, with mesangial area widening. Median survival of all patients was 24.4 months after diagnosis. The estimated 1,2,3 year survival rate was (65 ± 10 )%, (46 ± 12 )% and (15 ± 12 )% respectively. There was significant difference in median survival between the two groups (24.7 months in the group of 14 patients with isolated kidney affected,16.4 months in the group of 11 patients with kidney and other organs involved,P = 0.03). By univariate analysis, kidney associated with other organs amyloidosis and renal dysfunction were relevant to prognosis (P ＜ 0.05) and heart involvement was probably relevant (P = 0.06),whereas sex,age,plasma cell ratio,serum albumin level and hemoglobin level had no relation(P＞ 0.05 ). Multivariate analysis revealed that renal dysfunction at the time of diagnosis was a significant and independent prognostic factor for survival (P ＜0.05). Conclusions Renal dysfunction at the time of diagnosis is the best predictor of survival. The presence of amyloidosis in organs other than the kidney, such as advanced cardiac amyloidosis, predicts a poor survival.