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[原发性累及心肺系统的轻链型淀粉样变性——2例报告]

[Amyloid light-chain amyloidosis with primary involvement of the cardiorespiratory system--2 case reports].

作者信息

Danzig V, Danzigová Z, Lukás K, Ort J, Elleder M, Campr V, Sikulová S

机构信息

II. interní klinika 1. LF UK, Praha.

出版信息

Vnitr Lek. 1994 Feb;40(2):104-7.

PMID:8140757
Abstract

The authors describe the cases of two women suffering from AL amyloidosis with affection of the heart muscle in particular. In one of the patients there were also massive amyloid deposits in the lungs which is an exceptional finding. The observations confirm the adverse prognosis of amyloidosis, the rapid progression of the disease after the onset of symptoms of cardiac failure. The first cardial manifestation in both patients were palpitations. The first patient, but not the second one, had a myeloma confirmed on necropsy.

摘要

作者描述了两名患有AL淀粉样变性病且心肌受累的女性病例。其中一名患者肺部也有大量淀粉样蛋白沉积,这是一个罕见的发现。这些观察结果证实了淀粉样变性病的不良预后,即心力衰竭症状出现后疾病进展迅速。两名患者的首发心脏表现均为心悸。第一名患者在尸检时确诊患有骨髓瘤,而第二名患者没有。

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