浆细胞异常相关心脏淀粉样变性的诊断与治疗。

Diagnosis and Treatment of Cardiac Amyloidosis Related to Plasma Cell Dyscrasias.

机构信息

Division of Cardiovascular Medicine, Stanford Amyloid Center, Stanford University School of Medicine, Stanford, CA, USA; Stanford Cardiovascular Institute, Stanford University School of Medicine, Stanford, CA, USA; Advanced Heart Failure and Transplant Cardiology, Stanford University School of Medicine, 1651 Page Mill Road, Room 2300, Palo Alto, CA 94305, USA. Electronic address: https://twitter.com/KMAlexanderMD.

Stanford Cardiovascular Institute, Stanford University School of Medicine, Stanford, CA, USA; Stanford University School of Medicine, 1651 Page Mill Road, Room 2300, Palo Alto, CA 94305, USA.

出版信息

Cardiol Clin. 2019 Nov;37(4):487-495. doi: 10.1016/j.ccl.2019.07.013.

DOI:10.1016/j.ccl.2019.07.013

PMID:31587789

Abstract

Light chain amyloidosis is a deadly disease in which a monoclonal plasma cell dyscrasia produces misfolded immunoglobulin light chains (AL) that aggregate and form rigid amyloid fibrils. The amyloid deposits infiltrate one or more organs, leading to injury and severe dysfunction. The degree of cardiac involvement is a major driver of morbidity and mortality. Early diagnosis and treatment are crucial to prevent irreversible end-organ damage and improve overall survival. Treatment of AL cardiac amyloidosis involves eliminating the underlying plasma cell dyscrasia with chemotherapy and pursuing supportive heart failure management.

摘要

轻链淀粉样变是一种致命的疾病，其中单克隆浆细胞异常产生错误折叠的免疫球蛋白轻链（AL），这些轻链聚集并形成刚性的淀粉样纤维。淀粉样沉积物浸润一个或多个器官，导致损伤和严重功能障碍。心脏受累程度是发病率和死亡率的主要驱动因素。早期诊断和治疗对于预防不可逆的终末器官损伤和提高总体生存率至关重要。AL 心脏淀粉样变性的治疗包括用化疗消除潜在的浆细胞异常，并进行支持性心力衰竭管理。