Valenzuela-Rodríguez Germán, Araoz Tarco Ofelia, Fernández Vertiz Iván
Clínica Delgado, Lima, Peru. Clínica Delgado Lima Peru.
Unidad de Revisiones Sistemáticas y Metaanálisis (URSIGET), Vicerrectorado de Investigación, Universidad San Ignacio de Loyola, Lima, Peru. Universidad San Ignacio de Loyola Unidad de Revisiones Sistemáticas y Metaanálisis (URSIGET) Vicerrectorado de Investigación Universidad San Ignacio de Loyola Lima Peru.
Arch Peru Cardiol Cir Cardiovasc. 2025 Jun 27;6(2):104-109. doi: 10.47487/apcyccv.v6i2.477. eCollection 2025 Apr-May.
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.
我们报告一例系统性轻链淀粉样变性伴心脏受累病例，其之前存在单克隆IgG λ型丙种球蛋白病。临床诊断基于心力衰竭体征、心脏生物标志物升高以及特征性影像学表现。血液和尿液中游离轻链水平升高，以及脐周脂肪和胃肠道多个节段存在淀粉样沉积物，证实了诊断。开始使用达雷妥尤单抗、硼替佐米和地塞米松进行治疗，诊断后22个月进行自体造血干细胞移植，取得了良好的临床结果。
