Monoclonal gammopathy progressing to systemic amyloidosis with cardiac involvement. A case report.

Germán Valenzuela-Rodríguez, Ofelia Araoz Tarco, Iván Fernández Vertiz
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引用次数: 0

Abstract

We report a case of systemic light chain amyloidosis with cardiac involvement, preceded by a monoclonal IgG lambda gammopathy. The clinical diagnosis was based on signs of heart failure, elevated cardiac biomarkers, and characteristic imaging findings. The diagnosis was confirmed by increased levels of free light chains in blood and urine, as well as the presence of amyloid deposits in periumbilical fat and multiple segments of the gastrointestinal tract. Treatment with daratumumab, bortezomib, and dexamethasone was initiated, followed by autologous hematopoietic stem cell transplantation 22 months after diagnosis, resulting in a favourable clinical outcome.

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单克隆伽玛病进展为累及心脏的系统性淀粉样变性。一份病例报告。
我们报告一例全身轻链淀粉样变性与心脏累及,之前单克隆IgG λ γ病。临床诊断是基于心衰的迹象,心脏生物标志物升高,和特征性影像学发现。血液和尿液中游离轻链水平升高,脐周脂肪和胃肠道多节段出现淀粉样蛋白沉积,证实了诊断。开始使用达拉单抗、硼替佐米和地塞米松治疗,在诊断22个月后进行自体造血干细胞移植,获得了良好的临床结果。
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来源期刊
CiteScore
0.40
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审稿时长
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