摘要
免疫球蛋白轻链淀粉样变性(AL-淀粉样变性)的特点是骨髓浆细胞产生单克隆免疫球蛋白λ或κ轻链增生,这些不溶性蛋白沉积在组织器官引起组织器官结构损害及功能障碍。传统治疗(马法兰和肾上腺皮质激素)效果差,血液学缓解率较低。近年来自体造血干细胞移植和新的化学治疗方案提高了AL-淀粉样变性治疗的血液学缓解率和延长了生存时间,为该病的治疗带来新希望。该文重点就近年来AL-淀粉样变性的治疗进展作一介绍。
Immunoglobulin light chain （AL）amyloidosis is characterized by a clonal population of bone marrow plasma cells that produces a monoclonal light chain ofλorκtype. This insoluble protein deposits in tissues and interferes with organ function. Conventional treatment of AL-amyloidosis （melphalan and corti-costeroids） rarely resulted in hematologic complete response. In recent years,autologous stem cell transplanta-tion and some new chemical treatments resulted in higher hematologic response rates and survival ratea when compared with conventional treatment. This article mainly reviewed the progresses of treatments.
出处
《新医学》
2014年第1期1-4,共4页
Journal of New Medicine
关键词
AL-淀粉样变性
传统治疗
自体造血干细胞移植
化学治疗
Immunoglobulin light chain amyloidosis
Conventional treatment
Autologous stem cell transplantation
Chemical treatment
