Mejhert Marit, Hast Robert, Sandstedt Benngt, Janczewska Izabella
Department of Clinical Sciences, Ersta Hospital and Karolinska Institutet, Danderyd Hospital, Stockholm, Sweden.
BMJ Case Rep. 2011 Aug 17;2011:bcr0320114007. doi: 10.1136/bcr.03.2011.4007.
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.
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。
