Shimojima Yasuhiro, Matsuda Masayuki, Ishii Wataru, Koyama Jun, Yamamoto Kanji, Shimodaira Shigetaka, Sakashita Kazuo, Koike Kenichi, Ikeda Shu-ichi
Third Department of Medicine, Shinshu University School of Medicine, Matsumoto, Japan.
Intern Med. 2005 May;44(5):484-9. doi: 10.2169/internalmedicine.44.484.
A patient with primary systemic AL amyloidosis achieved partial hematological response after 2 courses of VAD (vincristine, doxorubicin and dexamethasone) and subsequent high-dose melphalan followed by autologous peripheral blood stem cell transplantation despite involvement of multiple organs, including the heart. In this patient natriuretic peptides and free light chains in serum were useful as markers of cardiac involvement and therapeutic effects, respectively. When amyloidosis-related dysfunction is seen in multiple organs, this intensive chemotherapy might be a possible therapeutic option, although several modifications in the regimen and careful management are necessary.
一名原发性系统性 AL 淀粉样变性患者,尽管多个器官包括心脏受累,但在接受 2 个疗程的 VAD(长春新碱、阿霉素和地塞米松)治疗以及随后的大剂量美法仑治疗并进行自体外周血干细胞移植后,获得了部分血液学缓解。在该患者中,血清中的利钠肽和游离轻链分别作为心脏受累和治疗效果的标志物。当在多个器官出现淀粉样变性相关功能障碍时,尽管需要对治疗方案进行一些调整并进行仔细管理,但这种强化化疗可能是一种可行的治疗选择。