YOSHIZAWA Seiichiro, AKAHANE Daigo, FUJIMOTO Hiroaki, GOTOH Moritaka, KIGUCHI Toru, TAUCHI Tetsuzo, OHYASHIKI Kazuma
Internal Medicine (Hematology/Oncology), Tokyo Medical University.
Rinsho Ketsueki. 2009 Nov;50(11):1647-51.
A 60-year-old woman was admitted with acute heart failure and was diagnosed as having primary systemic AL amyloidosis with cardiac involvement by endomyocardial biopsy. Electrophoresis revealed an IgG-lambda monoclonal component and amyloidosis was evident in the gastric and rectal mucosa. Her cardiac function at diagnosis was poor, including an ejection fraction of 59% and IVS of 19 mm, and serum cardiac troponin T (cTnT) was elevated (0.12 ng/ml). She was treated with melphalan-dexamethasone (Mel-Dex) therapy once a month. After more than a year, cardiac function and performance status were maintained, with decreasing levels of cTnT, indicating that Mel-Dex represents a feasible and effective therapeutic option for patients with AL amyloidosis with cardiac dysfunction.
一名60岁女性因急性心力衰竭入院，经心内膜心肌活检诊断为原发性系统性AL淀粉样变性伴心脏受累。电泳显示有IgG-λ单克隆成分，胃和直肠黏膜有明显的淀粉样变性。诊断时她的心脏功能较差，射血分数为59%，室间隔厚度为19mm，血清心肌肌钙蛋白T（cTnT）升高（0.12ng/ml）。她每月接受一次美法仑-地塞米松（Mel-Dex）治疗。一年多后，心脏功能和功能状态得以维持，cTnT水平下降，表明Mel-Dex是治疗伴有心脏功能障碍的AL淀粉样变性患者的一种可行且有效的治疗选择。
