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伴有良性单克隆丙种球蛋白病的原发性心血管淀粉样变性

Primary cardiovascular amyloidosis with benign monoclonal gammopathy.

作者信息

Pajor L, Kelényi G

出版信息

Virchows Arch A Pathol Anat Histopathol. 1984;403(2):195-203. doi: 10.1007/BF00695235.

Abstract

A 54-year-old woman is reported whose primary amyloidosis was diagnosed at autopsy. Amyloid deposits were found in the myocardium, the striated muscles, the smooth muscle layers of the gut and the wall of the blood vessels. The deposits showed resistance to induced proteolysis. A large number of mature plasma cells was demonstrated in the bone marrow, and immunocytochemical studies revealed a considerable increase in the proportion of plasma cells which were positive for kappa light chains of immunoglobulins, indicating a monoclonal gammopathy. This view was strongly supported by the unexpected finding that amyloid deposits were positive for kappa light chains. The relationship between the kappa positive reaction of amyloid and its resistance to induced proteolysis are discussed.

摘要

据报道,一名54岁女性的原发性淀粉样变性在尸检时被诊断出来。在心肌、横纹肌、肠道平滑肌层和血管壁中发现了淀粉样沉积物。这些沉积物对诱导性蛋白水解具有抗性。骨髓中显示出大量成熟浆细胞,免疫细胞化学研究表明,免疫球蛋白κ轻链呈阳性的浆细胞比例显著增加,提示存在单克隆丙种球蛋白病。淀粉样沉积物κ轻链呈阳性这一意外发现有力地支持了这一观点。本文讨论了淀粉样物质κ阳性反应与其对诱导性蛋白水解的抗性之间的关系。

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