Edinburgh Heart Centre, Royal Infirmary of Edinburgh, Edinburgh, UK
The University of Edinburgh, Edinburgh, UK.
BMJ Case Rep. 2021 Feb 18;14(2):e238499. doi: 10.1136/bcr-2020-238499.
A 53-year-old man presented to a district general hospital with chest pain, ECG changes and a small high-sensitivity cardiac troponin I rise. There were no symptoms of heart failure. CT coronary angiography revealed moderate calcific disease and conventional angiography confirmed no flow limitation. Echocardiography showed left ventricular hypertrophy (LVH). His blood pressure remained normal throughout his admission. The tertiary centre labelled this as a 'plaque rupture' event but the LVH remained unexplained. Cardiac MRI displayed an unusual pattern of late gadolinium enhancement, which was not classical of amyloid. However, a raised serum free kappa light chain along with the deposition of amyloid on his bone marrow aspirate confirmed the diagnosis of primary AL amyloidosis with cardiac involvement. The patient went on to have chemotherapy and remained stable at 1-year follow-up.
一位 53 岁男性因胸痛、心电图改变和小幅度高敏心肌肌钙蛋白 I 升高到地区综合医院就诊。他没有心力衰竭的症状。CT 冠状动脉造影显示中度钙化病变，常规血管造影证实无血流受限。超声心动图显示左心室肥厚（LVH）。他在整个住院期间血压保持正常。该三级中心将此标记为“斑块破裂”事件，但 LVH 仍未得到解释。心脏 MRI 显示出一种不同寻常的晚期钆增强模式，这不符合淀粉样变的经典表现。然而，血清游离 κ 轻链升高，骨髓抽吸物中有淀粉样蛋白沉积，这确诊了伴有心脏受累的原发性 AL 淀粉样变性。患者随后接受了化疗，在 1 年随访时仍然稳定。
