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首页> 外文会议>International Symposium on Amyloidosis >HIGH DOSE THERAPY WITH AUTOLOGOUS HEMATOPOIETIC STEM CELL TRANSPLANTATION (HSCT) FOR PATIENTS WITH PRIMARY SYSTEMIC AMYLOIDOSIS (AL): AN ABMTR STUDY
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HIGH DOSE THERAPY WITH AUTOLOGOUS HEMATOPOIETIC STEM CELL TRANSPLANTATION (HSCT) FOR PATIENTS WITH PRIMARY SYSTEMIC AMYLOIDOSIS (AL): AN ABMTR STUDY

机译：具有自体造血干细胞移植（HSCT）的高剂量治疗用于初级全身淀粉样蛋白症（AL）的患者：ABMTR研究

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摘要

Primary systemic amyloidosis (AL) is a rare plasma cell dyscrasia with approximately 2000 cases diagnosed yearly in North America. In this disease, monoclonal immunoglobulin light chains are produced by malignant plasma cells resulting in amyloid fibril deposition in vital organs leading to progressive, fatal multi-system failure. The median survival for treated patients is approximately 18 months compared to 12 months for untreated patients; the median survival for patients with symptomatic cardiac involvement is 6 months (1).With the promising outcomes observed with high dose chemotherapy and autologous hematopoietic stem cell transplantation in the more common plasma cell dyscrasia, multiple myeloma, pilot trials to test this approach in AL were pursued. These pilot trials, which almost uniformly utilized high dose melphalan, again based upon the experience in multiple myeloma, showed encouraging results: hematologic response rates were appreciated in approximately 60% of the patients; organ response rates were also observed but at a lower rate. Unfortunately, these early pilot trials were associated with high transplant-related mortality: 15-25%, and significantly higher, approaching 65% in patients with AL cardiomyopathy. More recent clinical trials have been conducted with more stringent patient selection criteria: transplant-related mortality has decreased to 10-15% (2).
机译：原发性淀粉样变（AL）是一种罕见的血浆细胞病有大约2000个病例北美每年诊断。在这种疾病中，单克隆免疫球蛋白轻链通过导致淀粉样蛋白原纤维沉积在重要器官导致进行性，致命多系统衰竭恶性浆细胞中产生。用于治疗的患者的中位生存是大约18个月相比为未经治疗的患者12个月;患者的症状的心脏介入的中位生存期为6个月（1）。随着以高剂量化疗和在更常见的浆细胞恶液质的自体造血干细胞移植，多发性骨髓瘤中观察到的有前途的结果，试试验，以测试在AL这种方法被追赶。这些中试试验，几乎均匀地利用高剂量美法仑，再次基于在多发性骨髓瘤的经验，表明了令人鼓舞的结果：血液学反应率的患者大约60％被理解;器官反应率也观察到，但以较低的速率进行。不幸的是，这些早期的试点试验用高移植相关死亡率相关：15-25％，而显著较高，接近患者65％的AL型心肌病。最近的临床试验已经用更严格的病人选择标准进行：移植相关死亡率已下降到10％-15％（2）。

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