Efficiency Of The Regiment Of Thalidomide Combined With Dexamethasone For The Treatment Of Immunoglobulin Light Chain Systemic Amyloidosis

Objective:Systemic immunoglobulin light chain amyloidosis(AL)is the most common type of amyloidosis with the worst prognosis.Chemotherapy and autologous stem cell transplantation are the main therapies for the disease.Foreign small sample researches showed the regiment of thalidomide combined dexamethasone was effective for recurrent and refractory AL patients,but there is no study about the regiment of thalidomide combined dexamethasone showing the efficacy and adverse reactions for untreated AL patients.The article retrospectively analyzes the clinical and prognostic dates of AL patients to evaluate the efficiency and safety of the regiment of thalidomide combined with dexamethasone in untreated patients of systemic immunoglobulin light chain amyloidosis.Methodology:The clinical dates of 63 patients diagnosed AL amyloidosis here were retrospectively analyzed from April 2009 to December 2014,all the patients took the regiment of thalidomide combined with dexamethasone as the first line treatment,all the them took the regiment for 4 cycles at least.The initial dose of thalidomide was 50 mg every night with increment to 100 mg after one week if there was no adverse reaction,and the highest dose was 200mg every night,the initial dose of dexamethasone was 40mg every week,one cycle of the treatment was 28 days.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.Result:A total of the 63 patients were enrolled in this study,the rate of males and females were 1.51:l,median age was 57,the cycle of treatment for 63patients is 19(4-78),38 patients had heart involved,and 38 patients had two more organs involved.In total,37 patients(58.7%)achieve different hematological response,median time of response were 4 months,in which 18 patients(28.6%)achieve complete response,median time of response were 3 months,10 patients(15.9%)achieve very good partial response,median time of response were 5.5 months and 9 patients(14.3%)achieve partial response,median time of response were 5 months.There were 23 patients(35.9%)obtaining organ response,of which mainly were renal response,median time to organ response were 8.5 months,heart response were(15.9%),median time to organ response were 18.8 months.3 patients had a relapse,median time to relapse was 7 months(3-19months).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 is worse comparing to the non-heart involved group.Common adverse reaction was sensoryneuropathy(23.8%),digestive tract reaction(17.5%),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 first line treatment of AL amyloidosis,it can be a candidate for patients were not suitable for high-dose melphalan/autologous peripheral blood stem cell transplantation or chemotherapy regiment of the bortezomib combined dexamethasone,and the long-term efficacy is still needed to be seen.