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[轻链沉积所致非淀粉样变性肾小球疾病:一例报告]

[Non-amyloidotic glomerular disease caused by light-chain deposits: a case report].

作者信息

Cantillo Jorge de Jesús, López Rocío del Pilar, Andrade Rafael Enrique

机构信息

Servicio de Nefrología y Diálisis, Hospital El Tunal, E.S.E., Bogotá, D.C, Colombia.

出版信息

Biomedica. 2009 Dec;29(4):531-8.

Abstract

The nephropathy of monoclonal gammopathies is principally caused by light chain deposits of fragmented immunoglobins. Paraprotein-related renal diseases are associated with such deposits of intact (heavy chain) or fragmentary (light chain) immunoglobins. A condition of pathological light chain deposits is rare and characterized by deposits of fragments of monoclonal immunoglobulins in many organs. Renal deposits occur primarily in glomeruli and tubular basement membranes. This disease is frequently associated with lymphoproliferative disorders. The majority of cases are caused by deposits of kappa light chains. Whereas this disease is most frequently associated with hematologic malignancies, occasionally a case occurs without detectable hematological pathologies; these cases are called idiopathic or primary. They usually manifest themselves as severe renal insufficiencies with nephrotic-range proteinuria. No treatment regime has been clearly established and the prognosis is poor. Herein, the clinical and histological characteristics are described regarding the first case in Colombia of light chain deposit disease without symptoms of malignancy.

摘要

单克隆丙种球蛋白病的肾病主要由片段化免疫球蛋白的轻链沉积引起。副蛋白相关的肾脏疾病与完整(重链)或片段化(轻链)免疫球蛋白的此类沉积有关。病理性轻链沉积的情况罕见,其特征是单克隆免疫球蛋白片段在许多器官中沉积。肾脏沉积主要发生在肾小球和肾小管基底膜。这种疾病常与淋巴增殖性疾病相关。大多数病例由κ轻链沉积引起。虽然这种疾病最常与血液系统恶性肿瘤相关,但偶尔也会出现无明显血液学病变的病例;这些病例被称为特发性或原发性。它们通常表现为伴有肾病范围蛋白尿的严重肾功能不全。尚未明确确立治疗方案,预后较差。在此,描述了哥伦比亚首例无恶性肿瘤症状的轻链沉积病的临床和组织学特征。

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