Imamura Tomoyuki, Ogata Masao, Kohno Kazuhiro, Tomo Tadashi, Ohtsuka Eiichi, Kikuchi Hiroshi, Kadota Jun-ichi
Division of Pathogenesis and Disease Control, Department of Infectious Diseases, Oita, Japan.
Am J Hematol. 2006 Apr;81(4):281-3. doi: 10.1002/ajh.20544.
No established treatments for systemic AL amyloidosis have been determined, and only four reports have described allogeneic stem cell transplantation for this disease. We report the case of a patient with orthostatic hypotension, diarrhea, nephrotic syndrome, and cardiac amyloidosis due to systemic AL amyloidosis. Reduced intensity allogeneic stem cell transplantation (RIST) was performed using a conditioning regimen comprising fludarabine 125 mg/m2 and melphalan 90 mg/m2. Hematologically complete remission and symptomatic improvement were obtained without severe transplantation-related complications. RIST may thus offer a useful treatment strategy for systemic AL amyloidosis complicated by cardiac amyloidosis.
目前尚未确定系统性 AL 淀粉样变性的既定治疗方法，仅有四份报告描述了针对该疾病的异基因干细胞移植。我们报告了一例因系统性 AL 淀粉样变性导致体位性低血压、腹泻、肾病综合征和心脏淀粉样变性的患者。采用包含氟达拉滨 125 mg/m² 和美法仑 90 mg/m² 的预处理方案进行了减低强度异基因干细胞移植（RIST）。获得了血液学完全缓解和症状改善，且无严重的移植相关并发症。因此，RIST 可能为并发心脏淀粉样变性的系统性 AL 淀粉样变性提供一种有效的治疗策略。
