单药贝兰他单抗马福多汀治疗复发性系统性AL淀粉样变性的实际疗效 系统性轻链淀粉样变性（systemic light chain amyloidosis, AL amyloidosis）是一种罕见的疾病，由单克隆免疫球蛋白轻链（light chain, LC）异常折叠并形成淀粉样纤维沉积在组织和器官中而导致。由于临床表现复杂且无特异性，AL amyloidosis 往往容易误诊和...
8.Kastritis E, Leleu X, Arnulf B, et al. Bortezomib, melphalan, and dexamethasone for light-chain amyloidosis [J]. J Clin Oncol, 2020, 38 (28): 3252-3260. 9.Kastritis E, Dialoupi I, Gavriatopoulou M, et al. Primary treatment of light-chain amyloidosis with bortezomib, lenalidomide,...
Light chain amyloidosis needs to be considered in the differential diagnosis of patients with nondiabetic nephrotic range proteinuria, unexplained fatigue or heart failure with preserved ejection fraction, a progressive peripheral neuropathy associated with a monoclonal protein, or atypical multiple myeloma. ...
AL型淀粉样变性多见于老年人，诊断中位年龄60岁左右，普遍存在诊断延迟，中国患者的平均延迟时间11.3±10.4个月①。AL淀粉样变性临床表现多样，受累器官众多。路瑾提到，在我国确诊患者中，最常见的受累器官是心脏和肾脏，七成以上患者出现了多脏器受累。因此，AL型淀粉样变性患者经常被误诊或在病程后期发现，严重影响...
淀粉样变性是一种浆细胞疾病(表1)。免疫球蛋白轻链淀粉样变性(immunoglobulin light chain amyloidosis,AL)即AL型淀粉样变性,为最常见的系统性淀粉样变性。 目前认为AL型淀粉样变性的发病主要与异常增生的浆细胞或B细胞克隆产生异常免疫球蛋白轻链——淀粉样物质前体...
8.Hou HA, Tang CH, Goh CH, et al. A population-based cohort study of the epidemiology of light-chain amyloidosis in Taiwan [J]. Sci Rep, 2022, 12 (1): 15736. 9.Gillmore JD, Wechalekar A, Bird J, et al. BCSH Committee. Guidelines on the diagnosis and investigation of AL amyl...
1. Liu Y, Leung N, Lu J. Systemic light chain amyloidosis: the hope for a cure.Sci Bull (Beijing) . 2023 Oct 16:S2095-9273(23)00714-4. doi: 10.1016/j.scib.2023.10.014. EM-144906 Content Approved Date:11/13/2023 仅...
Treatment of light chain (AL) amyloidosis with the combination of bortezomib and dexamethasone [J]. Haematologica, 2007, 92 (10): 1351-1358. 6.Venner CP, Lane T, Foard D, et al. Cyclophosphamide, bortezomib, and dexamethasone therapy in AL amyloidosis is associated with high...
原发性轻链型淀粉样变(Primary Light Chain Amyloidosis,Amyloid light-chain Amyloidosis,AL)是系统性淀粉样变性最常见的形式,是一种由具有反向β折叠结构的单克隆免疫球蛋白轻链沉积在器官组织内,并造成相应器官组织功能异常的系统性疾病,发病男性多于女性。
Amyloid light chain (AL) amyloidosis is among the more common and more severe of the amyloidoses usually involving the slow proliferation of a bone-marrow-residing plasma cell (PC) clone and the secretion of unstable immunoglobulin-free light chains (FLC) that infiltrate peripheral tissues and res...