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肾脏疾病组织病理学图谱:轻链沉积病

Histopathological atlas of renal diseases: light chain deposition disease.

作者信息

Ferrario Franco, Rastaldi Maria Pia

机构信息

Renal Immunopathology Center, S. Carlo Borromeo Hospital and Nuova Nefrologia Research Association, Milan--Italy.

出版信息

J Nephrol. 2005 Sep-Oct;18(5):499-502.

Abstract

Monoclonal diseases of B-cell lineage, often referred to as plasma cell dyscrasias, are characterized by abnormal and uncontrolled proliferation of a single clone of B cells at different maturation stages, with a more or less marked differentiation to immunoglobulin (Ig)-secreting plasma cells. Thus B-cell proliferation is usually associated with the production and secretion in blood of a monoclonal Ig or a fragment thereof. An ominous consequence of secretion of monoclonal Ig products is their deposition in tissue. These proteinaceous deposits can take the form of casts (in myeloma cast nephropathy), cristals (in myeloma-associated Fanconi's syndrome), fibrils (in light-chain [LC] amyloidosis), or granular precipitates (in monoclonal Ig deposition disease [LCDD]).

摘要

B细胞系单克隆疾病,通常称为浆细胞发育异常,其特征是单个B细胞克隆在不同成熟阶段异常且不受控制地增殖,并或多或少明显分化为分泌免疫球蛋白(Ig)的浆细胞。因此,B细胞增殖通常与血液中一种单克隆Ig或其片段的产生和分泌有关。单克隆Ig产物分泌的一个不祥后果是它们在组织中的沉积。这些蛋白质沉积物可以呈管型(在骨髓瘤管型肾病中)、晶体(在骨髓瘤相关的范科尼综合征中)、纤维(在轻链[LC]淀粉样变性中)或颗粒状沉淀物(在单克隆Ig沉积病[LCDD]中)的形式。

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