Charliński Grzegorz, Jurczyszyn Artur, Wiktor-Jedrzejczak Wiesław
Przegl Lek. 2014;71(2):102-9.
Immunoglobulin (Ig) light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of Ig light chain are deposited in tissues. Clinical features depend on organs involved but can include restrictive cardiomyopathy, nephrotic syndrome, hepatic failure, peripheral/ autonomic neuropathy. The diagnosis can be challenging, requiring a biopsy and often specialized testing to confirm the subtype of systemic disease. The goal of treatment is eradication of the monoclonal plasma cell population and suppression of the pathologic light chains which can result in organ improvement and extend patient survival. Standard treatment approaches include high dose melphalan (HDM) followed by autologous hematopoietic stem cell transplantation (SCT) or oral melphalan with dexamethasone (MelDex). The use of novel agents (thalidomide, lenalidomide and bortezomib) alone and in combination with steroids and alkylating agents has shown efficacy and continues to be explored.
免疫球蛋白（Ig）轻链淀粉样变性是一种克隆性、非增殖性浆细胞疾病，其中Ig轻链片段沉积于组织中。临床特征取决于受累器官，但可包括限制性心肌病、肾病综合征、肝功能衰竭、周围/自主神经病变。诊断可能具有挑战性，需要进行活检且常常需要专门检测以确认全身性疾病的亚型。治疗目标是根除单克隆浆细胞群体并抑制病理性轻链，这可使器官状况改善并延长患者生存期。标准治疗方法包括大剂量美法仑（HDM）随后进行自体造血干细胞移植（SCT）或美法仑联合地塞米松口服（MelDex）。单独使用新型药物（沙利度胺、来那度胺和硼替佐米）以及与类固醇和烷化剂联合使用已显示出疗效，并且仍在继续探索。
