一名患有免疫球蛋白轻链（AL）淀粉样变性且心脏、肝脏和胃肠道有沉积物的患者自体干细胞移植后的十年随访。
Ten-year follow-up after autologous stem cell transplantation of a patient with immunoglobulin light-chain (AL) amyloidosis with deposits in the heart, liver and gastrointestinal tract.
作者信息
Mejhert Marit, Hast Robert, Sandstedt Benngt, Janczewska Izabella
机构信息
Department of Clinical Sciences, Ersta Hospital and Karolinska Institutet, Danderyd Hospital, Stockholm, Sweden.
出版信息
BMJ Case Rep. 2011 Aug 17;2011:bcr0320114007. doi: 10.1136/bcr.03.2011.4007.
Abstract
The prognosis in amyloid light chain (AL)-amyloidosis and multiorgan involvement is poor, with a high-treatment-related mortality after high-dose melphalan and autologous stem cell transplantation (HDM/SCT). Some patients, however, might benefit from the therapy. We report a case of cardiac AL-amyloidosis with multiorgan involvement where the progressive cardiomyopathy was halted after successful treatment with HDM/SCT in 2001. The patient is in an excellent cardiac condition with a good quality of life, receiving treatment with angiotensinogen receptor blockers and a flexible diuretics regimen at follow-up after 10 years.
摘要
淀粉样轻链（AL）淀粉样变性合并多器官受累的预后较差，在接受大剂量美法仑和自体干细胞移植（HDM/SCT）后，与治疗相关的死亡率很高。然而，一些患者可能会从这种治疗中获益。我们报告一例心脏AL淀粉样变性合并多器官受累的病例，该患者在2001年成功接受HDM/SCT治疗后，进行性心肌病得到缓解。该患者心脏状况极佳，生活质量良好，在随访10年后，接受血管紧张素原受体阻滞剂治疗，并采用灵活的利尿剂方案。
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