轻链沉淀病肾脏损害1例报道及文献回顾

Renal impairment in light chain depositiondisease :one case report with review of liter-ature

报告了１例轻链沉淀病（ＬＣＤＤ）合并多发性骨髓瘤（ＭＭ）具有典型肾脏损害的患者。临床表现为肾病综合征、肾功能损害、异常浆细胞增生、低γ血症等。肾活检病理示肾小球系膜结节、肾小管基膜增厚、免疫酶标法抗κ轻链阳性。ＬＣＤＤ与异常浆细胞增生有关，多合并ＭＭ，肾脏损害为其首发和突出表现，肾功能受累明显。肾脏病理有特异性的改变，免疫荧光或免疫酶标法可检出轻链的沉积，是明确诊断的重要依据。

ne case of light chain deposition dis-ease (LCDD ) accompanied by multiplemyeloma (MM ) with typial renal inipair-ment was reported. Clinically, the patienthad nephrotic syndrome, renal functiondamage , plasma cell dyscrasia and hypogammaglobulinemia. The renal pathology revealed mesangial nodules . thickening oftubular basement membrane and positive re-action to k light chain antiserum by im-munohistochemistry. It is shown in litera-ture that LCDD is almost alwavs associatedwith plasma cell dyscrasia and is commonlyaccompanied with MM. The patients usuallybegin with and mainly suffer from renal im-pairment. among which the renal functiondamage is prominent. LCDD has typicalpathological changes with deposition oflight chains detected by immunofluorescenceor immunohistochemistry. The diagnosis ismainly based on the pathological findings.