Abstract:

Ｏｂｊｅｃｔｉｖｅ：To investigate the midterm curative effect of autologous hematopoietic stem cell transplantation (AHSCT) for patients with primary systemic amyloidosis (AL).
Ｍｅｔｈｏｄｏｌｏｇｙ：A total of eighty seven patients diagnosed as AL and received AHSCT were enrolled in this study from July 2010 to March 2014. They were 46 males and 41 females with an average age of 519±780 years old. The mean time of follow up was 21 months ranged from 12 to 33 months, and their midterm curative effects of AHSCT were analyzed.
Ｒｅｓｕｌｔｓ：All patients had achieved in AHSCT, the common complication were digestive tract symptoms during AHSCT, the incidence of grade 3 and above complications was low. At 12 months, 24 months and 36 months after AHSCT, the OS rate was 943%, 839% and 789%; PFS rate was 851%, 835% and 835%; Kidney survival rate was 917%, 826% and 771%, respectively. Transplantation related mortality (TRM) was 34%. Multivariate analysis showed that brain natriuretic peptide (BNP)  and difference free light chain (dFLC)＞5807 mg/L were independently associated with patient survival. Urinary N Acetyl beta Glucosidase D (NAG) was independently associated with kidney survival.
Ｃｏｎｃｌｕｓｉｏｎ：AHSCT was effective and safe for selected AL patients, the hematologic and organ response rate was high, and the OS and kidney survival rate were also exciting.