Winship Cancer Institute, Emory University, 1365C Clifton Road, Atlanta, GA, 30322, USA.
Curr Hematol Malig Rep. 2018 Jun;13(3):212-219. doi: 10.1007/s11899-018-0450-1.
Light-chain-associated (AL) amyloidosis is a rare disease with a poor prognosis. However, we have made recent strides in more accurate diagnosis and effective treatment. Here, we discuss the most recent updates and advancements during the past year in the diagnosis, prognostication, and management of AL amyloidosis both in the upfront and relapsed setting.
New imaging modalities, such as cardiac magnetic resonance (CMR) and use of fluorine-labeled radiotracers, are emerging as an important diagnostic tool in conjunction with biomarkers in the diagnosis, prognosis, and monitoring of the effects of therapy. In addition, ongoing evaluation of plasma cell-directed therapeutics, including daratumumab, pomalidomide, and ixazomib, as well as promising targeted novel therapies, such as the monoclonal antibody NEOD001, are in development. In conclusion, incorporating the use of plasma cell-directed therapy and novel agents targeting the amyloid deposits itself hold enormous potential in achieving improved outcomes in AL amyloidosis.
轻链相关性(AL)淀粉样变性是一种预后不良的罕见疾病。然而,我们在更准确的诊断和有效治疗方面取得了最新进展。在此,我们讨论了过去一年中在 AL 淀粉样变性的初始和复发治疗中,在诊断、预后和管理方面的最新进展。
新的成像方式,如心脏磁共振(CMR)和使用氟标记放射性示踪剂,与生物标志物一起,作为一种重要的诊断工具,在诊断、预后和监测治疗效果方面具有重要意义。此外,正在评估针对浆细胞的治疗药物,包括达雷妥尤单抗、泊马度胺和伊沙佐米,以及有前途的靶向新型治疗药物,如单克隆抗体 NEOD001,都在研发中。总之,浆细胞靶向治疗的应用和针对淀粉样沉积物本身的新型药物具有巨大的潜力,可以改善 AL 淀粉样变性的预后。