| 免疫球蛋白轻链淀粉样变性的治疗进展 |
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| 引用本文： | 马峰,孙世仁.免疫球蛋白轻链淀粉样变性的治疗进展[J].新医学,2014(1):1-4. |
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| 作者姓名： | 马峰 孙世仁 |
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| 作者单位： | 第四军医大学西京医院肾脏内科, 西安710032 |
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| 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。
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| 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 |
Progression of treatment on immunoglobulin light chain amyloidosis |
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| MA Feng,SUN Shi-ren.Progression of treatment on immunoglobulin light chain amyloidosis[J].New Chinese Medicine,2014(1):1-4. |
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| Authors: | MA Feng SUN Shi-ren |
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| Institution: | ( Xifing Hospital of the Fourth Military Medical University Nephrology, Xi＇an 710032, China) |
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| Abstract: | 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. |
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| Keywords: | Immunoglobulin light chain amyloidosis Conventional treatment Autologous stem cell transplantation Chemical treatment |
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