Villesuzanne Camille, Jaccard Arnaud, Nicol Martin
Service d'immuno-hématologie, hôpital Saint-Louis, AP-HP, Paris, France.
Service d'hématologie clinique et de thérapie cellulaire, centre de référence amylose AL et autres maladies par dépôt d'immuno- globulines monoclonales, centre hospitalier universitaire de Limoges, France.
Rev Prat. 2020 Dec;70(10):1137-1141.
Al amyloidosis, from diagnosis to treatment. AL amyloidosis is a rare hemopathy characterized by immunoglobulin light chains deposits in almost all organs causing organ failure. The main issue is the early dia¬gnosis, which must be made in front of an unexplained non-specific symptomatology, especially cardiac or renal, in frequently elderly patients with monoclonal gammo¬pathy. Non-invasive biopsies should be made for histolo¬gical confirmation revealing positive congo red and birefringent yellow-green deposits in polarized light specific for amyloidosis. Severity is assessed by biologi¬cal markers of cardiac involvement. The treatment consists in eliminating the plasma or lympho-plasma cell dyscrasia secreting the amyloidogenic light chain, and in proposing supportive care specific to this pathology.
AL淀粉样变性：从诊断到治疗。AL淀粉样变性是一种罕见的血液病，其特征是免疫球蛋白轻链沉积于几乎所有器官，导致器官功能衰竭。主要问题在于早期诊断，对于经常出现单克隆丙种球蛋白病的老年患者，在出现无法解释的非特异性症状，尤其是心脏或肾脏症状时，必须进行早期诊断。应进行非侵入性活检以进行组织学确认，显示刚果红阳性以及在偏振光下呈现淀粉样变性特有的双折射黄绿沉积物。通过心脏受累的生物学标志物评估疾病严重程度。治疗包括消除分泌淀粉样生成轻链的浆细胞或淋巴浆细胞异常增生，并提供针对该病理状况的支持性治疗。
