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題名 | 類澱粉樣蛋白病變之治療=Management of Primary Amyloidosis |
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作者 | 許育栽; 王郁青; 鄭奕帝; 張展維; 林季伶; Hsu, Yu-tsai; Wang, Yu-chin; Cheng, Yih-dih; Chang, Chan-wei; Lin, Chi-ling; |
期刊 | 藥學雜誌 |
出版日期 | 20130300 |
卷期 | 29:1=114 2013.03[民102.03] |
頁次 | 頁123-128 |
分類號 | 415.13 |
語文 | chi |
關鍵詞 | 澱粉樣蛋白病變; 單株漿細胞增生失調性疾病; Primary amyloidosis; Plasma cell dyscrasia; |
中文摘要 | 類澱粉樣蛋白病變 (primary amyloidosis) 是由於單株漿細胞增生失調性疾病所引起的,不溶性的纖維免疫球蛋白輕鏈沉積在身體造成器官衰竭甚至是死亡,發生率為約為每年每百萬人有8人。未經治療之病人其中位存活期為13個月,10年以上之長期存活率小於5%。類澱粉樣病變的首選治療為 melphalan 加上 prednisolone 或 dexamethasone,其中位存活期分別為29個月及5.1年。高劑量的靜脈注射 melphalan 合併自體造血幹細胞移植可以用來治療類澱粉樣蛋白病變,並且有良好的完全反應率,但不幸的由於治療過程的相關死亡率較高,因此只有約20%的病人符合篩選標準可接受自體造血幹細胞移植。此篇文章在於探討澱粉樣蛋白病變的相關病理學、臨床表徵、治療與文獻證據支持的療法。 |
英文摘要 | Primary amyloidosis results from a plasma cell dyscrasia in which insoluble fibrillar light chain protein deposition leads to organ failure and death with incidence of 8 cases per million population per year. The median survival of untreated patients is 13 months from the time of diagnosis. The long-term survival (>10 years from diagnosis) of untreated patients with primary amyloidosis is less than 5%. An oral regimen of melphalan and steroid (prednisone or dexamethasone) has been recommended as the standard treatment for primary amyloidosis and the median survival is 29 months and 5.1 years respectively. Moreover, high-dose intravenous melphalan with autologous stem cell transplantation (HDM/ASCT) can treat primary amyloidosis and results in a high complete response rate, but this is limited eligibility due to the risk of procedural morbidity and mortality with probably only 20% of cases being genuinely low risk from the treatment of ASCT, as per stringent criteria. This article attempts to describe for the pathology, clinical characteristics, management and evidence based to support the treatment of primary amyloidosis. |
本系統之摘要資訊系依該期刊論文摘要之資訊為主。