A Study On Incidence, Survival Rate And Prognostic Factors Of Myelodysplastic Syndrome And Aplastic Anemia In Adults

ObjectiveMyelodysplastic syndrome(MDS) and aplastic anemia are common blood diseases. We aimed at describing the epidemiology of these diseases,including the incidence, survival rate, prognosis, and summarizing the clinical data about treatment of 142 cases with AA and 435 cases with MDS in order to improve the level of treatment in adults in Shanghai.Methods1.We selected the cases of MDS and AA in adults new diagnosed in 2004-2006 in Jing'an District, Xuhui District, Huangpu District, Changning District, Putuo District and Yangpu District of Shanghai(China) to calculate the incidence.It is a retrospective study.2.Four hundred and thirty-five patients diagnosed as primary MDS in Sino-US Leukemia Cooperative Group of Shanghai were studied prospectively to identify the prognostic factors by Log-rank test and COX regression model.3.We analyzed the clinical characteristics and treatment of 142 cases with AA in Sino-US Leukemia Cooperative Group of Shanghai.These cases were followed up about 2-6 years.It is a prospective cohort study.Result1.Between 2004 and 2006, a total of 169 cases of MDS were identified in adults in six districts of Shanghai.The overall incidence was 1.45 per 100000 per year. The incidence of 18-34 age,35-59, age and≥60 age was 0.34,1.01 and 3.75 per 100000 per year, respectively. A total of 38 cases of aplastic anemia were identified in adults. The overall incidence was 0.33 per 100000 per year. The incidence of 18-34 age,35-59, age and≥60 age was 0.40,0.14 and 0.64 per 100000 per year, respectively.The incidence of SAA was 0.17 per 100000 per year, while the incidence of NSAA was 0.13 per 100000 per year.2.The 3-year survival rate of MDS was 46.7%. The median survival time of refractory cytopenia with multilineage dysplasia (RCMD) subtype was 38 months, RAEB subtype was 10 months.Four hundred and twenty-four patients have successful karyotype.According to IPSS score, low risk, intermediate risk-Ⅰ, intermediate risk-Ⅱand high risk group accounted for 38/424 (9.0%),282/424 (66.5%),74/424(17.5%)and 30/424 (7.1%).Multivariate analysis of COX model showed old age, lower neutrophil absolute count (NAC), lower hemoglobin, lower platelet count and IPSS group were independent factors associated with overall survival time (OS).WHO classification of prognostic scoring system(WPSS) is not an independent prognostic factor. But for cases with RCMD, platelet count, IPSS score, IPSS cytogenetics group and chromosome abnormality were not independent prognostic factors.Whereas age (≥60 years old), NAC(<1.0×109/L), Hb (<90g/L)and number of cytopenias were independent factors.A new score system was estabished based on these factors. It has better ability to predict OS for RCMD patients proved by univariate and multivariate analysis.3.Only 12% severe AA patients were treated with antithymocyte globulin (ATG) and cyclosporine A (CSA).Response rate was 78%,5-year survival rate was 89%. About 45% severe AA was treated with CSA alone. Response rate was 59%, 5-year survival rate was 76%. About 25% severe AA was treated with androgen alone. Multivariate analysis of COX model showed platelet count and treatment were independent factors associated with OS.ConclusionsThe incidence of MDS is higher than before, and the incidence is raised by age. The incidence of severe aplastic anemia is consistent with the past. The survival rate and median survival time of MDS was similar to that in Western. IPSS score can predict prognosis for total MDS patients and it is better than WPSS.But both of them have no validity for RCMD subtype. New score system can distinguish the different prognosis for RCMD patients. The standard therapy (ATG combined CSA) should be popularized in Shanghai area.