Abstract:

Ｏｂｊｅｃｔｉｖｅ：To evaluate the efficiency and safety of the regiment of thalidomide combined with dexamethasone in untreated   primary systemic (AL) amyloidosis patients.
Ｍｅｔｈｏｄｏｌｏｇｙ：The clinical data of 63 patients diagnosed AL amyloidosis were retrospectively analyzed from April 2009 to December 2014. All of them were taken the regiment of thalidomide combined with dexamethasone as the first line treatment. Survival curves were constructed according to the KaplanMeier method. The relation of clinical factors to allcause mortality was assessed using proportional hazards regression.
Ｒｅｓｕｌｔｓ：They were 38 males and 25 females with a median age of 57 ys. The cycle of treatment  was 19 (4~78). 38 patients had heart involved, and 38 patients had also two more organs involved. In total, 37 patients (587%) achieve different hematological response, median time of response was 4 months; in which 18 patients (286%) achieve complete response, median time of response was 3 months; 10 patients (159%) achieve very good partial response, median time of response was 55 months;  and 9 patients (143%) achieve partial response, median time of response was 5 months. There were 23 patients (365%) obtaining organ response, in which mainly was renal response, median time to organ response was 85 months, heart response rate were 159%, median time to organ response was 188 months. 3 patients had a relapse, median time to relapse was 7 months (3~19 months).The median followup period was 28 months, the survival rate at two years was 785%, and the median overall survival time did not reach. Compared to the nonhematological response group, the hematological response group has a better prognosis, prognosis of the heart involvement group was worse comparing to the non-heart involved group. Common adverse reaction was sensory neuropathy (238%), digestive tract reaction (174%), asthenia (111%), rash (63%) and so on, without treatment related mortality. 9 patients ended treatment for the adverse effects, dose of thalidomide was decreased in 12 patients, and dose of dexamethasone was decreased in 22 patients.Ｃｏｎｃｌｕｓｉｏｎ：The regiment of thalidomide combined with dexamethasone seems to be an effective and tolerant approach for the firstline treatment of AL amyloidosis, longterm efficacy is still needed to be observed.

Key words: AL amyloidosis, thalidomide, dexamethason, eefficiency of , treatment, adverse reaction