Amyloidosis Research and Treatment Center, Department of Molecular Medicine, Fondazione IRCCS Policlinico San Matteo and University of Pavia, Pavia, Italy.
Clin Lymphoma Myeloma Leuk. 2013 Apr;13(2):244-6. doi: 10.1016/j.clml.2013.02.001. Epub 2013 Mar 7.
Amyloidosis associated with immunoglobulin M clones is a distinct clinical entity that poses specific challenges to clinicians. Although there is substantial overlap, the pattern of organ involvement is peculiar, with higher frequencies of lung, lymph nodes, and peripheral nervous system involvement. Early diagnosis is vital to start effective therapy before irreversible organ damage has occurred and should be based on markers of initial, asymptomatic organ dysfunction, such as natriuretic peptides for heart involvement and albuminuria for renal amyloidosis. Immunoglobulin M clones can give rise to both light chain (AL) and reactive (AA) amyloidosis, and once the diagnosis of amyloidosis is made, correct amyloid typing is necessary to design appropriate therapy and follow-up. Prognostic stratification should include serum albumin concentration, which is an independent prognostic factor.
免疫球蛋白 M 克隆相关淀粉样变性是一种独特的临床实体，给临床医生带来了特殊的挑战。尽管存在大量重叠，但器官受累的模式是特殊的，肺、淋巴结和周围神经系统受累的频率更高。早期诊断对于在不可逆的器官损伤发生之前开始有效的治疗至关重要，并且应该基于初始无症状器官功能障碍的标志物，例如心脏受累的利钠肽和肾脏淀粉样变性的蛋白尿。免疫球蛋白 M 克隆可引起轻链 (AL) 和反应性 (AA) 淀粉样变性，一旦做出淀粉样变性的诊断，就需要正确的淀粉样变分型来设计适当的治疗和随访。预后分层应包括血清白蛋白浓度，这是一个独立的预后因素。
