| Objective To investigate the clinical features of pure red cell aplasia (PRCA), and to evaluate the effectiveness of immunosuppressive therapy. Method Clinical data of inpatient cases since 1989 and outpatient casessince 2000 were collected. Follow-up information on therapy effect and prognosis was collected through interview, telephone call, and questionnaire.Result Totally, 82 cases were collected, among which, 59 cases werediagnosed as primary type, and 23 cases were determined to be secondary type. The average age of the patients was 53 years old. Follow-up was complete in 58 patients, among which, 12 patients were cured, 22 patients achieved a clinical remission, 6 patients were relieved, while 6 patients were refractory to all therapies and become transfusion dependent and 12 patients were dead.Conclusions Immunosuppressive therapy is effective for PRCA, andcyclo- sporine appears to be the most effective drug for the disease, showed a total response rate of 78.6%, and the response rate of prednisone was 43.6%. Combination of cyclosporine tripterygium glycosides showed a superior effect to cyclosporine monotherapy. Tripterygium glycosides is effective in some cases, and its usefulness is to be determined. Aggressive chemotherapy or more potent immunosuppressant may be helpful for refractory cases. |
