【摘要】 轻链淀粉样变性(light-chain amyloidosis,AL)即原发性系统性淀粉样变性,是由于浆细胞克隆性增殖引起轻链型淀粉样蛋白前体在不同组织中形成不溶性蛋白纤维沉积,可累及全身多个器官,引起多个器官功能衰竭。该病发病率低,临床表现复杂多变,误诊率高,常延误治疗时机,预后较差。现报道1例多器官累及AL,并结合文献复习,探讨该病的发病机制、诊断、治疗与预后特征,提高对该病诊断及治疗的认识,降低误诊率。更多还原

【Abstract】 Light-chain amyloidosis(AL) is a plasma cell dyscrasia characterized by the pathologic production of fibrillar proteins comprised of monoclonal light chains which deposit in tissues and can cause organ dysfunction. The diagnosis can be challenging because of the low incidence and complexity of clinical manifestations. To improve the understanding of the diagnosis and treatment of AL,the clinical features,pathogenesis,diagnosis,therapy and prognosis of AL were further investigated through literatures review.更多还原