The Clinical And Laboratory Futures Of Myelodysplastic Syndrome With Erythroid Hyperplasia

Objective:1.To explore the clinical and cytogenetic futures,and factors affectingoutcome of Myelodysplastic syndrome(MDS),according to the newest standard(2016 WHO).2.To investigate the clinical and cytogenetic characteristics of myelodysplastic syndrome with erythroid hyperplasia(MDS-erythroid),and comparison with erythroid hyperplasia<50%erythroid nucleated cells(ENCs)group(MDS with<50%ENCs).3.To find a better way of calculating the proportion of bone marrow(BM)blasts for disease classification and risk stratification compared for MDS-erythroid patients and all MDS patients.Methods:1.Retrospective analysis from January 2010 to November 2015,1193 MDS patients in our hospital and 135 cases of AEL patients based on the WHO(2008)classification.Following adjustment for the latest 2016 WHO criteria,we analysis of new divided 1283cases of patients with MDS,molecular and cytogenetic characteristics,evaluation of existing IPSS,WPSS and IPSS-R score is still applicable.2.to compare the clinical and biological characteristics of 346 patients of MDS-erythroid and 937 patients with erythroid proliferation<50%ENCs in MDS,and to compare the influence of different levels of red cell proliferation on prognosis.3.respectively using bone marrow progenitor cells/all nucleated cells(TNCs),bone marrow primitive cells/(non-erythroid nucleated cells)(NECs)to calculate the percentage of bone marrow blasts of two kinds of methods and discover advantages and disadvantages of two methods in disease classification and prognosis assessment.Result:1.A total of 346(27.0%,346/1283)patients with MDS-erythroid and 937(73.0%,937/1283)patients with MDS with<50%ENCs(control group)WHO fulfilled the WHO(2016)criteria were identified.Two-thirds(90/135)of acute erythroleukemia patients were reclassified as MDS due to BM blast proportions of<20%TNCs,?20%NECs,and?50%ENCs.These newly categorized MDS patients accounted for 26.0%(90/346)of the MDS-erythroid patients.The rest of the 256 patients(74.0%,256/346)had?50%ENCs and<20%BM blasts,and static NECs with MDS(MDS-static).In all MDS patients,the median age was 58 years old(range 1-92 years old),with63.8%patients older than 50 years.and the absolute neutrophil count at presentation was1.26×10~9/L(range 0.00~72.63×10~9/L).Hemoglobin levels at presentation were 72 g/L(range 10-167 g/L),and platelet counts were 52×10~9/L(range 1~1504×10~9/L).A total of544 patients(42.5%)were observed to have clonal chromosome abnormalities.The most common type of chromosome aberration was trisomy 8(182/1283,14.19%).Other aberrations,in order of frequency,were-7/del(7q)(105/1283,8.18%),-5/5q-(106/1283,8.26%),-20/del(20q)(84/1283,6.55%),and chromosomes 1(74/1283,5.77%).A total of 620MDS patients had a median follow-up time of 38 months(range 0.5-79months).According to prognosis of MDS subtypes:a better prognosis is MDS-SLD and MDS-U group,the mortality rate both at the end of the follow-up less than 50%;the worst survival for the MDS-EB-2 group,the median survival time was 14 months.IPSS-R(2012)scoring system for 2016 WHO type differentiation of MDS patients,and evaluate its effect is stronger than the IPSS and WPSS score system,multivariate analysis showed that peripheral blood hemoglobin levels,age and IPSS-R group is an independent prognostic factor in patients with MDS-erythroid,OS group of hemoglobin<100g/L,age more than 50 years old,IPSS-R group the relatively high risk group is lower than the tendency of more than 100g/L,patients aged<50,IPSS-R groups tend to relatively low risk.2.The median age of all 346 MDS-erythroid patients was 57 years old(range 13-89years old),with 61.0%patients older than 50 years.and the absolute neutrophil count at presentation was 1.23×10~9/L(range 0.00~72.63×10~9/L).Hemoglobin levels at presentation were 72 g/L(range 10-142 g/L),and platelet counts were 54×10~9/L(range 3-715×10~9/L).A total of 145 patients(41.9%)were observed to have clonal chromosome abnormalities,of which 52 patients possessed a complex karyotype.The most common type of chromosome aberration was trisomy 8(14.45%,50/346).Other aberrations,in order of frequency,were-7/del(7q)(9.54%),-20/del(20q)(8.96%),and-5/del(5q)(7.51%).The median survival time of MDS-erythroid was 40 months(0.5-79 months).The overall survival(OS)rate in the 3 year was 49%,and the 5 year is 42%.In addition,we compared the clinical and genetic characteristics of MDS-erythroid with MDS possessing<50%ENCs and found that there were no significant differences except for BM blast percentage(by TNCs,median,3.0%vs.4.0%;P=0.007;by NECs,median,8.0%vs.6.0%;P<0.001).Simultaneously,the OS by univariate analysis between MDS-erythroid patients and MDS patients with<50%ENCs had also no statistical difference(median OS,40.0 vs.36.0 months;P>0.05).The level of red cell proliferation in patients with 0~30%was worse than that of MDS-erythroid patients.3.By calculating the percentage of BM blasts from NECs,62.1%(215/346)of patients were re-classified into the higher-risk WHO categories from the initial types calculated from TNCs.A total of 215 upgraded patients had poor survival compared to the 131patients from the unchanged MDS-erythroid group(median OS,31 months vs.not reached,P=0.011).A total of 73 of 200 patients(36.5%)with MDS-erythroid were diagnosed by WHO subtypes without excess blasts(EB)were reclassified into the higher-risk categories by NECs rule.The OS of these 73 patients was significantly shorter than for the 127 patients WHOse WHO subtypes remained without excess blasts(EB)(median OS,31 months vs.not reached,P=0.038).A total of 40 of 168 MDS-erythroid patients(23.8%)in relatively low-risk categories(including very low,low and intermediate categories according to IPSS-R)were re-classified to higher-risk categories(including high and very high categories).Additionally,these patients had significantly shorter OS than patients WHO remained in the lower-risk categories(median OS,34 months vs.not reached;P=0.030),especially in the intermediate risk group as stratified by IPSS-R(median OS,not reached for unchanged patients vs.22 months for re-classified patients,P=0.007).Conclusion:1.the clinical features of patients with MDS classified according to 2016 WHO classification were consistent with those reported in previous cases of MDS in china.IPSS-R scoring system is still a powerful tool for prognostic analysis.2.MDS-erythroid is a group of erythroid hyperplasia active groups in MDS,and had similar clinical characteristics,cytogenetic features and overall survival as MDS with<50%ENCs.Meanwhile,compared with bone marrow erythroid hyperplasia level is0~30%,it hold a better survival.3.Counting bone marrow blasts as a percentage of non-erythroid cells improves the prognostic evaluation of MDS with erythroid predominance,but not in MDS patients.