Clinical Features Of Myelodysplastic Syndromes Patients With Autoimmune Disorders

Objective: Objective to explore the correlation between chronic inflammation and immune abnormalities and the pathogenesis of myelodysplastic syndrome(MDS)by analyzing the clinical features and immune index characteristics of patients with MDS combined with autoimmune abnormalities,and to elaborate the impact of immune dysfunction on the disease and its prognostic value.Methods: The clinical data of 142 patients with MDS who were first diagnosed in the second hospital of Lanzhou University from January 2012 to January 2020 with complete serum Immunology Examination or a history of autoimmune diseases(ADs)were collected.According to whether there were autoimmune abnormalities or ADs,the patients were divided into three groups:(1)the group with ADs(n=33);(2)the group with abnormal serum immune test without related symptoms(n=19,referred to as the abnormal serum immune test group);(3)the group without autoimmune abnormality(n=90,the control group).SPSS 22.0 statistical software was used to analyze the basic clinical features,serum EPO level,peripheral blood T lymphocyte subsets and related cytokines,chromosome karyotypes,gene mutation,2016 WHO subtybes,IPSS-R risk stratification,conversion to secondary acute myeloid leukemia and overall survival.And Cox regression analysis was used to analyze the risk factors of survival.Results: Among 142 patients with MDS,52(36.62%)had autoimmune abnormalities,33(23.24%)had confirmed ADs,and 19(13.38%)had only abnormal serum immunity.Compared with the non autoimmune abnormal group,the proportion of female,MDS-EB-1 subtype,IPSS-R score as medium high risk group was higher,serum EPO level was lower,the proportion of Th17 cells,IL-6,TNF-α,IFN-γ and IL-17 in peripheral blood were higher in ADs group(P<0.05);the proportion of patients with abnormal serum immune examination: the patients with higher median age and more than 60 years of age were higher,and only,the level of IL-6,IFN-γ inperipheral blood was higher(P<0.05),but there was no significant difference in gender,constituent ratio of 2016 WHO subtybes,IPSS-R prognosis group and conversion to secondary acute myeloid leukemia.There was no significant difference in the detection rate of chromosomal karyotypes abnormality and mutation frequency of TET2 and SF3B1 among the three groups,but TET2 had the highest mutation frequency in the group with ads,DNMT3 A had the highest mutation frequency in the abnormal serum immune test group,and the mutation frequency of U2AF1 and SF3B1 was the highest in the normal serum immune test group.There was no significant correlation between the abnormal chromosome karyotype,mutation gene and the related immunological indexes.The median survival time of patients with ADs and abnormal serum immune test was lower than that of patients without autoimmune abnormality(34.00 months,37.00 months vs 48.00 months,respectively),but the difference was not statistically significant(P=0.077,0.208,respectively).However,multivariate analysis showed that the presence of ADs was an independent poor prognostic factor of MDS(P< 0.05).Conclusion: The percentage of MDS with autoimmune abnormality was 36.62%.ADs was associated with MDS-EB-1 subtype,high IPSS-R risk stratification and poor prognosis.The changes of inflammatory and immune indexes in MDS with autoimmune abnormality were more obvious,suggesting that chronic inflammation,cellular immunity and humoral immunity were involved in the occurrence and development of MDS.This result has a certain reference value to guide the precise individualized treatment and prognosis of MDS.