Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine , Seoul , South Korea .
Amyloid. 2013 Dec;20(4):204-11. doi: 10.3109/13506129.2013.824417. Epub 2013 Aug 5.
Autologous stem cell transplantation (ASCT) has improved the prognosis of selected patients with immunoglobulin amyloid light-chain (AL) amyloidosis. However, there exist little data regarding Asian patients receiving ASCT. We retrospectively analyzed 24 patients who were treated with ASCT for AL amyloidosis between 2007 and 2012. The median age at the time of ASCT was 56 years (range: 40-62 years), and in 63% of patients, the AL amyloidosis involved two or more organs. All patients except one received induction treatment such as cyclophosphamide, dexamethasone or thalidomide before ASCT, but only approximately half of these patients showed a hematologic response. After ASCT, the overall hematologic response was increased to 92% (22/24), which translated into increased organ response in 58% of patients (14/24). There was no transplantation-related mortality (0%) even though cardiac amyloidosis patients were included in our series. In conclusion, our results of ASCT in patients with AL amyloidosis were comparable to that of Western countries in terms of response and survival outcomes. Therefore, ASCT is an effective and feasible treatment approach for Asian patients with AL amyloidosis.
自体干细胞移植 (ASCT) 改善了某些免疫球蛋白轻链 (AL) 淀粉样变性患者的预后。然而，关于接受 ASCT 的亚洲患者的数据较少。我们回顾性分析了 2007 年至 2012 年间接受 ASCT 治疗的 24 例 AL 淀粉样变性患者。ASCT 时的中位年龄为 56 岁（范围：40-62 岁），63%的患者 AL 淀粉样变性累及两个或更多器官。除 1 例患者外，所有患者均在 ASCT 前接受了环磷酰胺、地塞米松或沙利度胺等诱导治疗，但只有约一半的患者出现血液学反应。ASCT 后，总体血液学反应增加到 92%（22/24），58%（14/24）的患者器官反应增加。尽管我们的系列中包括了心脏淀粉样变性患者，但没有与移植相关的死亡率（0%）。总之，我们在 AL 淀粉样变性患者中进行 ASCT 的结果在反应和生存结果方面与西方国家相当。因此，ASCT 是治疗亚洲 AL 淀粉样变性患者的有效且可行的方法。
