摘要
目的:了解轻链沉积病(LCDD)肾损害的临床和病理特征.方法:回顾性分析26例经临床和肾活检确诊的LCDD患者的临床和病理改变.结果:26例LCDD患者起病时的平均年龄为49.4岁(27～72岁),其中男性21例,女性5例.8例确诊为多发性骨髓瘤(MM),2例伴有浆细胞异常增生,另有16例病因不明.临床表现为急性肾衰3例(11.5%),慢性肾衰17例(65.4%),肾病综合征17例(65.4%),15.4%的患者起病时即需行肾脏替代治疗.实验室检查发现血清和尿液游离κ、λ轻链的阳性率分别为56.5%和91.7%.肾脏病理改变以系膜结节样病变多见,占53.8%,2例表现为膜增生样病变、1例为膜性病变,中-重度小管间质慢性化病变为本组患者较特征性的病变(92.3%).肾组织中以λ轻链沉积为主者占65.4%(17例),κ轻链沉积占34.6%(9例).18例患者行肾组织电子显微镜检查,均显示肾小球和(或)肾小管基膜内(外)侧不规则的纤细颗粒样电子致密物.结论:LCDD患者临床以肾功能不全伴肾病综合征多见,部分患者合并浆细胞增生性疾病.病理以系膜结节样伴严重的小管间质病变为特征.血清、尿液以游离λ轻链增高多见,肾组织中也以λ轻链沉积居多.
Objective: To investigate the clinical and pathological features of patients with light-chain deposition disease (LCDD). Methodology: From April 1991 to January 2001, twenty-six patients with LCDD were reviewed retrospectively. The clinical and pathological feature of those patients has been analyzed. Eighteen of them were undergone electron microscopy examinations. Results: They were 21 male and 5 female, with an average age of 49.4 years (range 27 to 72). Eight patients were diagnosed myeloma, and two were abnormal plasma cellular proliferation, however, sixteen (69.2 %) had a monoclonal protein of undetermined significance without evidence of myeloma. Among 26 patients, 65.4% of them had renal dysfunction at presentation, followed by nephrotic syndrome (65.4%), chronic renal failure (53.8), acute renal failure (11.5%), asymptomatic proteinuria (3.8%). The typical glomerular lesion was a diffuse mesangial nodular lesion that was positive for periodic scid-Schiff (PAS) stain with acute and chronic tubuloinsterstitial change. Seventeen patients (65.4%) had l light-chain deposition and nine (34.6%) had k light-chain deposition. The free l and k light-chain demonstrated in the serum of 56.5 % and in the urine of 91.7% in our patients. In electron microscopy, the fine or coarse granular electron-dense deposits that delineated the outer aspect of the tubular basement membranes were observed. Conclusion: The most clinical syndrome of LCDD with renal lesion was renal failure with nephrotic syndrome. A monoclonal protein may never be found in either serum or urine in a majority of patients. The nodular glomerulosclerosis was the most frequent pathological change.
出处
《肾脏病与透析肾移植杂志》
CAS
CSCD
2003年第6期525-529,共5页
Chinese Journal of Nephrology,Dialysis & Transplantation
