Sanchorawala Vaishali
Amyloid Treatment and Research Program and Stem Cell Transplantation Program of Section of Hematology and Oncology, Boston Medical Center Boston, MA 02118, USA.
Am J Blood Res. 2012;2(1):9-17. Epub 2012 Jan 1.
AL amyloidosis is the most common form of systemic amyloidosis and is associated with an underlying plasma cell dyscrasia. The disease is often difficult to recognize because of its broad range of manifestations and, what are often, vague symptoms. Recent diagnostic and prognostic advances include the serum free light chain assay, cardiac magnetic resonance imaging, and serologic cardiac biomarkers. Treatment strategies that have evolved during the past decade are prolonging survival and preserving organ function in patients with this disease. This review outlines the role of high dose melphalan and stem cell transplantation in the treatment of AL amyloidosis.
AL淀粉样变性是系统性淀粉样变性最常见的形式,与潜在的浆细胞发育异常相关。由于其广泛的临床表现以及通常较为模糊的症状,该疾病常常难以识别。近期的诊断和预后进展包括血清游离轻链检测、心脏磁共振成像以及血清学心脏生物标志物。在过去十年中发展起来的治疗策略正在延长该疾病患者的生存期并保留器官功能。本综述概述了大剂量美法仑和干细胞移植在AL淀粉样变性治疗中的作用。