An accurate diagnosis of amyloidosis and its subtype classification are essential for disease prognostication and treatment. In primary amyloidosis, overall median survival is approximately 2 years and may be less in patients with cardiomyopathy. Current therapy for primary amyloidosis is suboptimal. Controlled studies suggest that treatment with melphalan and prednisone may provide marginal survival benefit. A more aggressive approach such as autologous hematopoietic stem cell transplantation may offer potential for long-term benefit. Although patients undergoing autologous hematopoietic stem cell transplantation are highly selected, response rates can approach 60%, and patients with amyloidosis who respond to treatment have potential for long-term survival. New treatment modalities that were shown to have antitumor activity in multiple myeloma (high-dose dexamethasone and thalidomide) may also be of therapeutic value in primary amyloidosis. Systemic chemotherapy would not be expected to have any beneficial effect on other forms of amyloid and carries significant risk.

中文翻译：

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免疫球蛋白轻链淀粉样变性的治疗：新的和旧的。

淀粉样变性及其亚型分类的准确诊断对于疾病的预后和治疗至关重要。在原发性淀粉样变性病中，总体中位生存期约为2年，患有心肌病的患者可能更少。当前用于原发性淀粉样变性的疗法不是最佳的。对照研究表明，用美法仑和泼尼松治疗可能提供边缘生存益处。诸如自体造血干细胞移植等更具侵略性的方法可能为长期受益提供潜力。尽管高度重视接受自体造血干细胞移植的患者，但缓解率可达到60％，对治疗有反应的淀粉样变性患者具有长期生存的潜力。在多发性骨髓瘤（大剂量地塞米松和沙利度胺）中显示具有抗肿瘤活性的新治疗方式也可能在原发性淀粉样变性病中具有治疗价值。预计全身化学疗法不会对其他形式的淀粉样蛋白产生任何有益作用，并且具有很大的风险。