Ueda Mitsuharu
Department of Neurology, Graduate School of Medical Sciences, Kumamoto University.
Brain Nerve. 2024 May;76(5):583-587. doi: 10.11477/mf.1416202647.
AL amyloidosis, derived from amyloidogenic immunoglobulin light chains, is a common type of systemic amyloidosis. Peripheral neuropathy has been identified in 10%-40% of patients with systemic AL amyloidosis. Definitive diagnosis requires tissue biopsies, including skin, fat, and gastrointestinal samples, as well as amyloid typing. Disease-modifying therapies have been shown to improve patient survival and prevent progressive organ dysfunction.
AL淀粉样变性由淀粉样免疫球蛋白轻链衍生而来,是系统性淀粉样变性的常见类型。10%-40%的系统性AL淀粉样变性患者已被确诊患有周围神经病变。明确诊断需要进行组织活检,包括皮肤、脂肪和胃肠道样本,以及淀粉样蛋白分型。已证明疾病修饰疗法可提高患者生存率并预防进行性器官功能障碍。