Clinical Analysis Of 15 Adult Patients With Acquired Pure Red Cell Aplasia

Objective:To analyze the clinical characteristics of adult acquired pure red blood cell aplastic anemia(PRCA),explore its pathogenesis and treatment plan,and improve the understanding of acquired PRCA.Methods:The medical records of 15 patients with acquired PRCA aged over 18 years who were admitted to our hospital from January 2011 to December 2022 were summarized,and the general clinical characteristics,laboratory examination characteristics,treatment plan and efficacy of the patients were analyzed retrospectively.Results:1.Among the 15 patients,6 were male and 9 were female.The age of onset ranged from 18 to 83 years old,with an average age of 63.7 years;2.In 15 cases of acquired PRCA,7 cases were primary and 8 cases were secondary.The etiology of secondary PRCA included T-LGLL,thymoma,MDS and r Hu EPO;3.At the initial diagnosis,hemoglobin(HGB)26-74g/L,reticulocyte percentage(Ret%)0.1%-0.46%,all<1%;Bone marrow smear: erythroid hyperplasia is reduced or absent,granulocyte/red ratio is inverted,granulocyte and megakaryocyte are normal;Flow cytometric immunity: abnormal expression of T lymphocytes was detected in 3patients at the initial diagnosis,and in 5 patients at the end of follow-up,all of them were associated with T-LGLL,and all of them were accompanied by increased proportion of CD3+and CD8+;Only one MDS patient was accompanied by chromosome karyotype abnormality;In the routine humoral immune function test,about half of the patients have abnormal immune function test;4.Of the 15 patients,3 patients with thymoma were treated with surgery and immunosuppressive therapy,and the other 12 patients were mainly treated with immunosuppressive therapy;The total reaction rate of 15 cases was 40%;5.Among the 15 patients,3 recurred during the treatment,which all occurred during the withdrawal or reduction of cyclosporine A,indicating that the recurrence of acquired PRCA was closely related to the maintenance treatment of cyclosporine A;Three cases died,except one case died of disease progression,the other two cases all died of its complicated serious infection.Conclusions:1.Adult acquired PRCA is more common in middle-aged and elderly people,with a low incidence rate and specific clinical manifestations and bone marrow smears;2.The etiology of acquired PRCA is complex,T-LGL and thymoma play an important role in secondary PRCA,and are often accompanied by abnormal immune function,especially almost all T-LGL-related PRCA can detect abnormal T lymphocytes;3.The treatment of adult acquired PRCA is mainly immunosuppressive therapy,with cyclosporine A and/or glucocorticoid as the first-line treatment scheme;The reduction or withdrawal of cyclosporine A is closely related to the recurrence and requires maintenance treatment;4.Patients with acquired PRCA have many adverse reactions after long-term immunosuppressive treatment,the most common of which is infection,especially lung infection.Some patients eventually died of severe infection.