轻链淀粉样变性累及多器官1例报道并文献复习

• 作 者: 朱耿军 沈卫章 任立参 金立方 贾姣源 连成 刘芳 邸鑫 王鹏 徐文
• 机 构: 吉林大学第二医院肿瘤血液科,首都医科大学附属北京地坛医院病理科,
• 出 处: 胃肠病学和肝病学杂志
• 发表日期: 2016年12期
• 关键词: 轻链淀粉样变性 硼替佐米 克隆性增殖 淀粉样蛋白前体 轻链型 误诊率 免疫固定电泳 沙利度胺 蛋白纤维 骨髓瘤细胞

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。

Light-chain amyloidosis (AL), the primary systemic amyloidosis, is a result of the clonal multiplication of plasma cells causing the formation of insoluble protein fibers in various tissues by light chain amyloid precursor Involves the body multiple organs, causing multiple organ failure. The incidence of the disease is low, the clinical manifestations of complex and volatile, misdiagnosis rate, often delay the timing of treatment, the prognosis is poor. It is reported in 1 case of multiple organ involvement AL, combined with the literature review to explore the pathogenesis of the disease, diagnosis, treatment and prognosis characteristics to improve the diagnosis and treatment of the disease awareness and reduce the misdiagnosis rate.