Witteles Ronald M, Liedtke Michaela
Division of Cardiovascular Medicine, Stanford Amyloid Center, Stanford University School of Medicine, Stanford, California, USA.
Division of Hematology, Stanford Amyloid Center, Stanford University School of Medicine, Stanford, California, USA.
JACC CardioOncol. 2019 Sep 24;1(1):117-130. doi: 10.1016/j.jaccao.2019.08.002. eCollection 2019 Sep.
AL amyloidosis results from clonal production of immunoglobulin light chains, most commonly arising from a clonal plasma cell disorder. Once considered a nearly uniformly fatal disease, prognosis has improved markedly over the past 15 years, predominantly because of advances in light chain suppressive therapies. Cardiac deposition of amyloid fibrils is common, and the severity of cardiac involvement remains the primary driver of prognosis. Improvements in chemotherapy/immunotherapy have prompted a reassessment of the role of advanced cardiac therapies previously considered contraindicated in most patients, including the role of implantable cardioverter-defibrillators and cardiac transplantation. This state-of-the-art review highlights the current state of the field, including diagnosis, prognosis, and hematologic- and cardiac-specific therapies.
AL淀粉样变性是由免疫球蛋白轻链的克隆性产生引起的，最常见于克隆性浆细胞疾病。曾经被认为几乎是一种 uniformly fatal disease（此处原文有误，应是“uniformly fatal disease”，意为“一致致命的疾病”），在过去15年里预后有了显著改善，主要是由于轻链抑制疗法的进展。淀粉样纤维的心脏沉积很常见，心脏受累的严重程度仍然是预后的主要驱动因素。化疗/免疫疗法的改进促使人们重新评估以前大多数患者被认为禁忌的高级心脏疗法的作用，包括植入式心脏复律除颤器和心脏移植的作用。这篇前沿综述强调了该领域的现状，包括诊断、预后以及血液学和心脏特异性疗法。
