Heart Care Partners, Wesley Hospital, Brisbane, Australia.
Heart Lung Circ. 2013 Aug;22(8):655-60. doi: 10.1016/j.hlc.2013.01.013. Epub 2013 Mar 7.
AL amyloidosis and multiple myeloma result in extracellular deposition of insoluble fibrillar protein in tissues and organs. Untreated median survival has been documented at 12 months. Cardiac infiltration decreases survival to five months. Chemotherapy and bone marrow transplantation (BMT) have been shown to improve survival when haematological remission is documented. This study aimed to assess if remission could result in cardiac structural improvement.
269 patients were treated with BMT for amyloidosis from 1997 to 2010. Cardiac amyloidosis was identified in 30 patients by echocardiographic criteria. Echocardiography was performed before and after BMT.
Thirteen of 30 patients with cardiac amyloidosis died during follow-up. No change in cardiac structure was seen in 11 patients. Average survival was 49 months from BMT for non-responders. Fifteen patients had cardiac normalisation (responders). The average time to normalisation was 25 months. Only two responders died. Average survival for responders was 71 months (p < 0.0001 compared with non-responders). Normalisation of cardiac structure was highly predictive of survival (Fisher's exact test p = 0.0025, relative risk 0.18).
Cardiac amyloidosis patients with haematological remission after chemotherapy and BMT may subsequently normalise cardiac structure and function. Normalisation is highly predictive of survival.
淀粉样变性和多发性骨髓瘤导致组织和器官中不溶性纤维状蛋白的细胞外沉积。未经治疗的中位生存期已记录为 12 个月。心脏浸润使生存期缩短至五个月。当血液学缓解得到证实时，化疗和骨髓移植（BMT）已被证明可改善生存。本研究旨在评估缓解是否会导致心脏结构改善。
1997 年至 2010 年，269 例患者因淀粉样变性接受 BMT 治疗。30 例心脏淀粉样变性患者通过超声心动图标准确定。在 BMT 前后进行超声心动图检查。
30 例心脏淀粉样变性患者中有 13 例在随访期间死亡。11 例患者的心脏结构无变化。无反应者的平均生存时间为 BMT 后 49 个月。15 例患者的心脏正常化（反应者）。平均正常化时间为 25 个月。只有两名反应者死亡。反应者的平均生存时间为 71 个月（p < 0.0001 与无反应者相比）。心脏结构正常化高度预测生存（Fisher 确切检验 p = 0.0025，相对风险 0.18）。
化疗和 BMT 后血液学缓解的心脏淀粉样变性患者随后可能会使心脏结构和功能正常化。正常化高度预测生存。
