Schulenburg A, Kalhs P, Oberhuber G, Reiter E, Base W, Greinix H T
Department of Medicine I, University of Vienna, Austria.
Bone Marrow Transplant. 1998 Aug;22(3):293-5. doi: 10.1038/sj.bmt.1701330.
The morbidity and mortality of AL amyloidosis is caused by the deposition of Ig light chains as amyloid protein in vital organs. With conventional therapy median survival of patients with AL amyloidosis is 10-14 months. With high-dose chemotherapy clinical remissions of organ-specific disease have been reported. Here, we present a patient with high-risk AL amyloidosis who was given high-dose therapy and a peripheral blood stem cell transplant. Four days later she died of gastrointestinal perforation due to amyloid infiltrations.
AL淀粉样变性的发病率和死亡率是由免疫球蛋白轻链作为淀粉样蛋白沉积在重要器官中所致。采用传统疗法时,AL淀粉样变性患者的中位生存期为10 - 14个月。据报道,采用大剂量化疗可使器官特异性疾病实现临床缓解。在此,我们报告一名高危AL淀粉样变性患者,其接受了大剂量治疗及外周血干细胞移植。四天后,她因淀粉样浸润导致胃肠穿孔而死亡。