Stefani Georgia, Kouvata Evangelia, Vassilopoulos George
Department of Hematology, Larisa University Hospital, 41110 Larisa, Greece.
Cell and Gene Therapy Lab, Biomedical Research Foundation of the Academy of Athens, 11527 Athens, Greece.
Life (Basel). 2023 Dec 26;14(1):42. doi: 10.3390/life14010042.
Light-chain amyloidosis (AL) is a disease of protean manifestations due to a wide spectrum of organs that can be affected. The disorder is caused by the deposition of an extracellular amorphous material, the amyloid, which is produced by malignant plasma cells. The latter usually reside in the bone marrow; plasma cell infiltration is often low, in sharp contrast to what we observe in multiple myeloma. The disease may run below the physician's radar for a while before clinical suspicion is raised and targeted tests are performed. In this short review, we try to answer most of the questions that a practicing physician may ask in a relative clinical setting. The text is formed as a series of reader-friendly questions that cover the subject of AL amyloidosis from history to current therapy.
轻链型淀粉样变性（AL）是一种临床表现多样的疾病，因为多种器官都可能受到影响。该疾病由细胞外无定形物质淀粉样蛋白沉积引起，淀粉样蛋白由恶性浆细胞产生。后者通常存在于骨髓中；与我们在多发性骨髓瘤中观察到的情况形成鲜明对比的是，浆细胞浸润往往较少。在临床怀疑并进行针对性检查之前，这种疾病可能会在一段时间内未被医生察觉。在这篇简短的综述中，我们试图回答执业医生在相关临床环境中可能提出的大多数问题。本文以一系列便于读者理解的问题形式呈现，涵盖了从AL淀粉样变性的病史到当前治疗的主题。
