Clinical Analysis Of 84 Cases Of Adult Patients With Acquired Pure Red Cell Aplasia In Multicenters

Objective:The aim of this study is to evaluate whether cyclosporine A(CsA)combined with corticosteroids(CS)is more effective than CsA or CS alone in the treatment of pure red cell aplasia(PRCA),and to determine whether patients will benefit from this treatment,as well as possible influencing factors,so as enhancing the understanding of the disease,improving the prognosis of the disease and increasing survival rate.Methods:Clinical characteristics and the treatment outcomes of 84 patients aged 18 years and over with acquired PRCA were collected from October2009 to June 2017 in the FirstAffiliated Hospital of Jilin University(37 cases)and the First Affiliated Hospital of Nanjing Medical University(36 cases)and Zhejiang Traditional Chinese Medicine Hospital(11 cases).The deadline for follow-up was June 2017(6 cases were lost to follow-up).All was performed using SPSS 20.Differences among variables were evaluated by the chi-square test or Fisher's exact test and t-test.A correlation analysis of relevant factors and efficacy was analyzed by the log-rank test.The overall survival rate was estimated by the Kaplan-Meier method.P values below 5% were considered statistically significant.Gender,age,primary and secondary,etiology of the patients were included in the analysis.The assessment ofpossible causes of PRCA included a previous history of drug use,toxins or infections,the liver and kidney function,immunological examination including auto-antibodies,T cell subsets,bone marrow examination including morphology,biopsy,cytogenetics(chromosome analysis).Polymerase chain reaction(PCR)was used to detect rearrangement of T cell receptor(TCR),and immunoglobulin heavy(IGH)chain gene and mutations in STAT3.The levels of TCRV? and killer cell inhibitory receptors(KIRs)in peripheral blood were assessed by flowcytometry,and the presence of thymoma and tumors was excluded by computed tomography.Results:Among 84 patients diagnosed as pure red cell aplasia,the CR rate and the response rate of CsA-containing group(CsA alone and the combination of CS and CsA)were better than CS alone(51.1% vs 15.8%,P<0.05,72.3% vs 36.8%,P<0.05).Combination of CS and CsA achieved better CR rate and response rate than CS alone(60% vs 15.8%,P<0.05,70% vs 36.8%,P<0.05).The response rate of CsA alone was better than CS alone(76.5% vs 36.8%,P<0.05),while CsA alone did not show better CR rate than CS alone(35.3% vs 15.8%,P>0.05).Time of CsA combined with CS to achieve complete remission was shorter than CS alone and CsA alone(P<0.05).There was no significant difference in age,gender,primary and secondary,and etiology.Conclusion:1.Adult patients with acquired pure red cell aplasia showed a good response to either corticosteroids or cyclosporine A.2.Combination of CS and CsA showed better CR rate and responserate than CS alone,and also produce remission faster than CS alone.We might choose CsA as the initial induction therapy in treating adult patients with acquired PRCA,and combination of CS and CsA could achieve better CR rate than CS alone.3.A maintenance therapy is important to prevent relapse,and a continuous and careful follow-up is required.