Clinical Features And Prognosis Of Amyloidosis And Evidence-based Analysis Of Treatment

Objective:To analyze the outcomes and clinical features of amyloidosis in xinjiang.And to evaluate the efficacy of autologous stem cell transplantation(ASCT)versus chemotherapyand ASCT in patients with immunoglobulin light chain(AL)amyloidosis using methodology recommended by the Cochrane Methods:114 patients biopsy-proven amyloidosis were included from January 2007 to January 2017 in xinjiang medical university,using Kaplan-Meier method to calculate the patient's survival rate,using COX regression model analysis related dangerous element.And to extract data by comprehensive literature search(Embase,Pubmed),and then used the Review Manager5.3 software for statistical analysis.Results: 114 patients were included in this study with the mean age was 54.07±13.26 years old diagnosis.They were 37 females.Edema and proteinuria was the major clinical feature of these patients and the kidneys(52.63%)and the heart(49.10%)were the most frequently affected organs.Patients with median overall survival time is 34.81 months,1 year,2 years,3 years,5 years survival rate is respectively:69.8%,62.2%,49.4% and 42.6%.The patients with kidneys involvement,heart involvement and more than two organs involvement had a worse survival,but the treatment can improve the prognosis of patients.COX multifactor analysis showed the creatinine,NYAH3-4 and treatment can significantly influence survival in these patients.By literature searching there were two RCT,five single-arm prospective studies,which were included in the analysis of 369 patients.Two RCT study compared with ASCT group,the hematologic remission rate,renal response,transplant related overall survival had no statistical significance(HR:1.41 [95%CI:0.72-2.77],P=0.32;HR:1.30 [95%CI:0.77-2.19],P=0.33;HR:0.76 [95%CI:0.54-1.07],P=0.12;HR:1.11 [95%CI:0.69,1.77],P=0.68).In the non-RCT studies,thechemotheapy and ASCT could improve the hematologic remission rate(OR:2.33 [95%CI:1.06-5.11],P=0.03);The renal reactionwas not statistically significant.(OR:1.36 [95%CI:0.91-2.05],P=0.13).Conclusion:Amyloidosis patients uaually combined with renal and heart involvement,the high level of serum creatinine and progression to cardiac failure implied bad outcomes,while receiving treatment may improve the prognosis of patients.Current clinical evidence show that compared with ASCT,chemotherapy and ASCT may improve hematologic remission rate,but the renal response,transplant related over all survival was indicating a need for well-designed andadequately powered RCTs to better address the role of ASCT in AL amyloidosis.