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Light Chain Amyloidosis 2014

机译:轻链淀粉样变性2014

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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.
机译:AL淀粉样变性病患者难以诊断。具有多器官尤其是心脏受累的患者被认为预后不良。因此,早期诊断至关重要。应根据风险给予治疗。自体干细胞移植的使用与高危患者的不可接受的毒性有关。低风险和一些中度风险的患者可能会受益于总体生存期延长的反应患者。新药,例如沙利度胺,来那度胺和硼替佐米,以及下一代IMiD和蛋白酶体抑制剂均来自多种骨髓瘤治疗方案。它们与地塞米松和烷化剂的组合即使在不适合移植的患者中也具有深远的影响。存活的患者即使在相当大比例的高风险,不良预后人群中也具有优异的无进展和总体存活率。鉴于近年来的进展,该评价包括2014年AL淀粉样变性患者治疗的最新动态。

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