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干燥综合征中λ型A的器官特异性结节性肺淀粉样变性

[Organ-specific nodular pulmonary amyloidosis of the A lambda type in Sjogren syndrome].

作者信息

Schneider F, Linke R P, Albrecht H J

机构信息

Rheumaklinik Oberammergau, Max-Planck-Institut für Biochemie Martinsried.

出版信息

Z Arztl Fortbild (Jena). 1997 Jan;90(8):719-23.

PMID:9133112
Abstract

The exclusive involvement of the lungs with A lambda-type amyloidosis in nodular dispositions in a case of Sjögren's syndrome is very rare. An immunoglobulin lambda-type light chain, benign, monoclonal gammopathy has been verified as the ethological cause. The urine concentration of paraproteins was below the detection limit of the common examination methods and could only be found immunoelectrophoretically in urine concentrated 100-200 fold. The question of a possible relationship with Sjögren's syndrome is being discussed.

摘要

在干燥综合征病例中,肺脏仅以结节状形式出现λ型淀粉样变性极为罕见。已证实病因是一种良性单克隆免疫球蛋白λ型轻链病。副蛋白的尿液浓度低于常规检测方法的检测限,仅在尿液浓缩100 - 200倍后通过免疫电泳才能检测到。目前正在讨论其与干燥综合征可能存在的关系问题。

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