| 免疫球蛋白轻链型淀粉样变性的诊疗进展 |
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| 引用本文： | 许利凡,李振宇. 免疫球蛋白轻链型淀粉样变性的诊疗进展[J]. 国际输血及血液学杂志, 2016, 0(3): 233-237. DOI: 10.3760/cma.j.issn.1673-419X.2016.03.009 |
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| 作者姓名： | 许利凡 李振宇 |
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| 作者单位： | 221002,江苏徐州医学院附属医院血液科 |
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| 基金项目： | 国家自然科学基金(81570183)，National Natural Science Foundation of China(81570183) |
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| 摘 要： | 免疫球蛋白轻链型淀粉样变性(AL)为最常见的系统性淀粉样变性,其特征为单克隆免疫球蛋白轻链的错误折叠引起的淀粉样蛋白的沉积.AL发病机制与多发性骨髓瘤(MM)相关,可继发于MM.目前,诊断延误仍然为影响AL疗效的最主要因素.因此,早期诊断及治疗对改善AL患者的预后具有重要意义.笔者拟就AL诊断、治疗及预后进行综述.
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| 关 键 词： | 淀粉样变性 诊断 药物疗法,联合 干细胞移植 预后 |
Diagnosis and treatment progress of immunoglobulin light chain amyloidosis |
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| Abstract: | Immunoglobulin light chain amyloidosis (AL),the most common of the systemic amyloidosis,is characterized by the deposition of amyloid fibrils that derives from the aggregation of misfolded monoclonal immunoglobulin light chains.Pathogenesis of AL is associated with multiple myeloma (MM),and AL can be secondary to MM.Currently delayed diagnosis is still the main obstacles to treatment of AL.Therefore,identifying early symptoms and early treatment are of great significance to improve the patient's prognosis.This article aims to analyze diagnosis,prognosis and treatment of AL. |
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| Keywords: | Amyloidosis Diagnosis Drug therapy,combination Stem cell transplantation Prognosis |
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