Department of Medicine, Division of Hematology, University of Miami/Sylvester Comprehensive Cancer Center, 1475 Northwest 12th Avenue, Miami, FL 33136, USA.
Boston University School of Medicine and Boston Medical Center, 72 East Concord Street, K-503, Boston, MA 02118, USA.
Hematol Oncol Clin North Am. 2020 Dec;34(6):1099-1113. doi: 10.1016/j.hoc.2020.08.004. Epub 2020 Sep 30.
Clinical features of soft tissue amyloid light-chain (AL) amyloidosis include macroglossia, arthropathy, muscle pseudohypertrophy, skin plaques, and carpal tunnel syndrome. Vascular manifestations of AL amyloid include periorbital ecchymosis, jaw or limb claudication, and even myocardial infarction caused by occlusion of small vessel coronary arteries. Some of these features, such as macroglossia, periorbital ecchymosis, and the so-called shoulder-pad sign, are pathognomonic for AL amyloidosis. These findings may be the initial presenting features of the disease, and the recognition of these red flag symptoms is very important for the diagnosis and early intervention on the underlying plasma cell disease.
软组织淀粉样轻链（AL）淀粉样变性的临床特征包括巨舌、关节病、假性肌肉肥大、皮肤斑块和腕管综合征。AL 淀粉样变性的血管表现包括眶周瘀斑、颌或肢体跛行，甚至由于小血管冠状动脉闭塞引起的心肌梗死。这些特征中的一些，如巨舌、眶周瘀斑和所谓的肩垫征，是 AL 淀粉样变性的特征性表现。这些发现可能是该疾病的初始表现特征，识别这些红旗症状对于诊断和早期干预潜在的浆细胞疾病非常重要。
