文章基本信息

标题：Light Chain Amyloidosis 2014
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作者：Moshe E. Gatt
期刊名称：International Journal of Hematological Disorders
出版年度：2014
卷号：1
期号：1A
页码：21-29
DOI：10.12691/ijhd-1-1A-4
语种：English
出版社：Science and Education Publishing
摘要：AL amyloidosis patients are difficult to diagnose. Patients having multi-organ and particularly cardiac involvement are considered to have bad prognosis. Early diagnosis is therefore essential. Treatment is to be given according to risk. The use of autologous stem cell transplantation is associated with unacceptable toxicity in high-risk patients. Low risk and some intermediate risk patients may benefit from it with responding patients having prolonged overall survival. New medications, such as thalidomide, lenalidomide and bortezomib, and next generation IMiDs and proteasome inhibitors are derived from multiple myeloma regimens. Their combination with dexamethasone and alkylating agents have a profound effect even in transplant ineligible patients.with surviving patients having excellent progression-free and overall survival, even in a significant proportion of high risk, poor prognosis populations. This review includes the latest updates on treatment for AL amyloidosis patients in 2014, in light of the progress done during the recent years.
关键词：Amyloidosis; therapy; novel- agents