Phase II trial of high-dose dexamethasone for untreated patients with primary systemic amyloidosis
Phase II trial of high-dose dexamethasone for untreated patients with primary systemic amyloidosis
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DOI：
10.1007/bf02785843
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发表时间：
1999-07-01
期刊：
影响因子：
3.4
通讯作者：
Kyle, RA
中科院分区：
文献类型：
--
作者：
Gertz, MA;Lacy, MQ;Kyle, RA
原发性系统性淀粉样变性是由不溶性免疫球蛋白轻链片段沉积引起的疾病。确诊后30天内就诊的患者中位生存期为13个月。接受美法兰和泼尼松治疗的患者中位生存期为17个月。需要新的治疗方法。对25例未经治疗的原发性全身性淀粉样变性患者采用大剂量地塞米松进行前瞻性研究。治疗方法与多发性骨髓瘤患者相似。在这一队列中，3例患者表现出客观回归，疾病的器官特异性改善。整个组的中位生存期为13.8个月。大剂量地塞米松对淀粉样变患者偶尔有益，但似乎并不优于美法兰和强的松化疗。
Primary systemic amyloidosis is the disorder that results from the deposition of insoluble immunoglobulin light chain fragments. Patients seen within 30 days of diagnosis have a median survival of 13 months. Patients treated with melphalan and prednisone have a median survival of 17 months. There is a need for new therapies. A prospective study was undertaken of high-dose dexamethasone in the treatment of 25 patients with previously untreated primary systemic amyloidosis. Treatment was similar to that given to patients with multiple myeloma. In this cohort, three patients showed objective regression with organ-specific improvement of the disease. The median survival of the entire group was 13.8 months. High-dose dexamethasone is of occasional benefit in patients with amyloidosis but does not appear to be superior to melphalan and prednisone chemotherapy.