Clinical Characteristics And The Efficacy Of Immunosuppressive Therapy Of 61 Patients With Acquired Pure Red Cell Aplasia

Objective: To retrospectively analyze the clinical features,laboratory features,immune status,treatment response and prognosis,evaluate the response of immunosuppressive therapy of adult acquired pure red cell aplasia patients,so as to understand the characteristics of the disease and improve the understanding of the disease.Methods: From December 2013 to may 2020,adult patients over 18 years old diagnosed as acquired pure red cell aplasia(PRCA)in the second hospital of Hebei Medical University were selected as the research objects.The clinical characteristics,treatment and prognosis of these patients were retrospectively analyzed.The sex,age,hemoglobin level,reticulocyte level,LDH,ferritin of primary and secondary patients were compared and studied.Spss25.0 software was used for statistical analysis.Results:1.A total of 61 patients with adult acquired PRCA were included,including 24 cases(39.3%)of primary PRCA and 37 cases(60.7%)of secondary PRCA.The most common secondary factors were connective tissue disease in 8 cases,thymoma related in 6 cases,and T-cell large granular lymphoblastic leukemia in 5 cases.The mean hemoglobin level was 55.23 ±13.75 g / L and the median reticulocyte percentage was 0.15%.There was no significant difference between primary PRCA and secondary PRCA in age,gender and hemoglobin level.2.The overall response rate(ORR)was 68.6%(35 / 51),the total CR rate was 39.2%(20 / 51),and the median onset time was 3.5(0.5-23)months.Among them,10 patients were treated with Glucocorticoid(Cs)alone,the overall response rate was 60.0%;12 patients were treated with cyclosporine A(CsA),the overall response rate was 75.0%;26 patients were treated with Cs and CsA,the overall response rate was 77.3%.The other 7 patients received other immunosuppressive regimens,including cyclophosphamide,methotrexate,rituximab,etc.only 3 patients achieved partial remission,and the overall response rate was 42.9%.There was no significant difference in the overall response rate among the four groups(60.0% vs 75.0% vs 77.3% vs42.9%,P = 0.336).However,the CR rate of CS combined with CsA was significantly higher than that of CsA alone and other immunosuppressive agents(63.3% vs 25.0%,P = 0.035,63.3% vs 0,P = 0.006).In addition,the ORR of primary PRCA was 63.2%,and that of secondary PRCA was 71.9%(P = 0.547).The response rate of primary PRCA treated with CsA was higher.There was no significant difference in response rate between different regimens of secondary PRCA.3.The ORR and CR of primary PRCA were 63.2% and 36.8%,respectively;The ORR and CR rate of secondary PRCA were 71.9% and40.6%,respectively.There was no significant difference in the initial response rate and CR rate between the two groups(P = 0.547,P = 0.514).Primary PRCA showed better ORR and CR rate in the treatment with CsA(P < 0.05),while secondary PRCA showed no significant difference in response rate between different treatment methods.But whether primary or secondary PRCA,CsA combined with Cs can achieve faster and higher CR.4.There are 16 patients failed to respond to the initial induction therapy.Among them,8 patients achieved CR or PR by changing the immunosuppressive therapy.The cumulative response rate was 84.3%(43 /51).34.3% of the patients relapsed at different times after remission of immunosuppressive therapy,and the median time of recurrence was 8 months(3-74 months).All the 12 patients relapsed because of the rapid withdrawal or reduction of immunosuppressive drugs.66.7% of the patients relapsed and could get effective response again according to the original treatment or by increasing the original treatment dose.CsA alone showed a lower recurrence rate(22.2%),but the difference was not statistically significant(P = 0.742).5.The most common complication was infection,accounting for 21.6%,and the most common one was pulmonary infection,accounting for 72.7%.6.The survival of patients was not related to gender,age,etiology and different treatment methods.Conclusions:1.This group of adult acquired pure red cell aplasia showed a good response rate to immunosuppressive therapy,mainly cyclosporine A and / or glucocorticoid.2.The response rate of patients with primary PRCA to CsA is better than that of patients with Cs,but whether it is primary or secondary PRCA,CsA combined with Cs can achieve faster and higher CR rate.Therefore,we recommend the combination of CsA and Cs as the initial induction of remission for adult acquired PRCA.3.The treatment of PRCA has a certain recurrence rate,and maintenance treatment with CsA is very important to prevent recurrence,and continuous and close follow-up is needed to reduce the incidence of adverse reactions.