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AL amyloidosis: new drugs and tests, but old challenges.

机译:AL淀粉样变性:新药和试验,但旧挑战。

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摘要

Immunoglobulin light chain (AL) amyloidosis develops in 2% of individuals with monoclonal plasma cell dyscrasias. In this issue of ONCOLOGY, Drs. Gertz and Dispenzieri discuss AL amyloidosis, highlighting progress in the field along with outstanding challenges.The authors present two cases that illustrate the frequent problem of late diagnosis that can arise when patients present with nonspecific clinical features. It is also possible that a lingering perception that amyloidosis is untreatable may discourage further diagnostic work-up, which generally requires biopsy confirmation, especially in the older patients among whom AL amyloidosis is most common. However, the authors emphasize that certain combinations of clinical features should trigger investigation for amyloidosis, including co-existent peripheral and autonomic neuropathy, cardiac failure in association with proteinuria or marked gastrointestinal symptoms, and left ventricular "hypertrophy" in the absence of a clear cause.
机译:免疫球蛋白轻链(AL)淀粉样变性病在2%的患有单克隆浆细胞异常的人中发展。在本期《肿瘤学》中,Dr。 Gertz和Dispenzieri讨论了AL淀粉样变性,重点介绍了该领域的进展以及面临的严峻挑战。作者提出了两个案例,说明了当患者表现出非特异性的临床特征时可能会出现的晚期诊断的常见问题。缠绵的淀粉样变性病无法治愈的认识也可能会阻碍进一步的诊断检查,这通常需要活检确认,特别是在AL淀粉样变性最常见的老年患者中。然而,作者强调,某些临床特征的组合应触发淀粉样变性的研究,包括并存的周围神经和自主神经病变,伴有蛋白尿或明显胃肠道症状的心力衰竭以及在没有明确原因的情况下左室“肥大” 。

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