Response to cyclosporine A and corticosteroids in adult patients with acquired pure red cell aplasia: serial experience at a single center

To assess response to cyclosporine A, and/or corticosteroids, and possible factors influencing the response in adult patients with acquired pure red cell aplasia (PRCA). Clinical data from 42 cases were retrospectively analyzed. These patients received cyclosporine A (CsA), and/or corticosteroids (CS), or other immunosuppressive agents on becoming refractory and relapse. Thirty-nine patients were evaluated. Remission induction therapy included CsA (n = 16), CS (n = 13), CsA in combination with CS (n = 6), or other immunosuppressive agents (n = 4). Initial response rates were 75.0, 46.2, 66.7 and 75.0%, respectively (P = 0.456). Cumulative response rates in patients who received CsA, CS, CsA in combination with CS, or other immunosuppressive agents were 69.6% (16/23), 50.0% (7/14), 71.4% (5/7), 42.9% (6/14), respectively (P = 0.376). Cumulative rates of CR were 26.1% (6/23), 28.6% (4/14), 57.1% (4/7), 14.3% (2/14), respectively (P = 0.284). In 27 refractory and relapsed PRCA patients, 11 of 17 patients (64.7%) achieved remission by CsA and/or CS regimen, while three of ten patients (30.0%) responded to other immunosuppressive agents (P = 0.120). CsA and/or CS were effective in treating PRCA. For patients with relapse or refractory PRCA, there were no satisfactory treatments if CsA and/or CS failed or were not administered.