Immunosuppressive Therapy For Adult Patients With Acquired Pure Red Cell Aplasia And Impact Factors Related To Efficacy

Objective: This article aims to evaluate whether the combination of cyclosporine A(CsA)and corticosteroids(CS)produce faster efficacy in acquired pure red cell aplasia(PRCA)than CsA alone or CS alone.Methods: Clinical data of 76 cases of PRCA were retrospectively collected from 2009 October.Initial therapy,efficacy,time to remission,relapse,factors impacted the clinical efficacy and death were also analyzed.Results: 70 patients were evaluated in two institutions(6 patients lost to follow-up).The remission induction therapy including CsA(n=20),CS(n=19),simultaneous combination of CsA and CS(n=31),achieved remission in 15/20(75.0%),8/19(42.1%),21/31(71.0%)patients,respectively.The CR rates and the response rates of CsA-containing group(CsA,CsA combined with CS)were better than CS(52.9% vs 21.1%,P=0.017;72.6% vs 42.1%,P=0.018).It showed no difference between CsA group and CS group in response rate and CR rate(75.0% vs 42.1%,P=0.054;40.0% vs 21.1%,P=0.301).Combination of CS and CsA achieved higher CR rate and response rate than CS(61.3% vs 21.1%,P=0.006;71.0% vs 42.1%,P=0.043).Time to achieve CR was shorter in group of CsA combined with CS than that in group of CS,or CsA(P=0.014).27 patients relapsed due to discontinuance or tapering therapy,and 19 patients regained response by increasing the dose of original regimens or changing to other immunosuppressive therapy.By univariate and multivariate analysis,CsA-containing group showed higher response rate than CS(P=0.004,P=0.022).By univariate analysis,CR rate was significantly different in age(P=0.035),sex(P=0.035)and regimen(CsA+CS vs CS)(P=0.01).By multivariate analysis,CsA combined with CS showed higher CR rate than CS(P=0.024),and idiopathic PRCA showed higher CR rate than secondary PRCA(P=0.023).Response to induction therapy was identified as correlative impact factor for death in PRCA(P=0.012).Conclusion: In treating adult patients with PRCA,combination of CS and CsA could improve the CR rate,and achieve CR faster.CsA combined with CS achieved higher CR rate than CS,and idiopathic PRCA achieved higher CR rate than secondary PRCA.Main reasons of relapse were discontinuance or tapering therapy.Response to induction therapy was a possible correlative impact factor for death in PRCA.