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.
KeywordsPure red cell aplasia Cyclosporine A Corticosteroids
Compliance with ethical standards
Conflict of interest
The authors declare that they have no conflict of interest.
Informed consent was obtained from all individual participants included in the study.
- 1.Dessypris EN, Lipton JM. Red cell aplasia. In: Wintrobe’s clinical hematology. 11th ed. Philadelphia: Lippincott Williams &Wilkins; 2004. p. 1421–1427.Google Scholar
- 2.Goldman L, Schafer AI. Aplastic anemia and related bone marrow failure states. In: Bagby GC, editor. Goldman’s cecil medicine. 24th ed. New York: Elsevier; 2011. p. 1083–90.Google Scholar
- 12.Sawada K, Hirokawa M, Fujishima N, Teramura M, Bessho M, Dan K, et al. Long-term outcome of patients with acquired primary idiopathic pure red cell aplasia receiving cyclosporine A. A nationwide cohort study in Japan for the PRCA Collaborative Study Group. Haematologica. 2007;92:1021–8.CrossRefPubMedGoogle Scholar
- 13.Hirokawa M, Sawada K, Nakao S, Fujishima N, Nakao S, Urabe A, et al. Long-term response and outcome following immunosuppressive therapy in thymoma-associated pure red cell aplasia: a nationwide cohort study in Japan by the PRCA collaborative study group. Haematologica. 2008;93:27–33.CrossRefPubMedGoogle Scholar
- 14.Dessypris EN. Pure red cell aplasia. Baltimore: Johns Hopkins University Press; 1988.Google Scholar
- 23.Fujishima N, Sawada K, Hirokawa M, Oshimi K, Sugimoto K, Matsuda A, et al. Long-term responses and outcomes following immunosuppressive therapy in large granular lymphocyte leukemia-associated pure red cell aplasia: a Nationwide Cohort Study in Japan for the PRCA Collaborative Study Group. Haematologica. 2008;93:1555–9.CrossRefPubMedGoogle Scholar
- 24.Hirokawa M, Sawada K, Fujishima N, Teramura M, Bessho M, Dan K, et al. Long-term outcome of patients with acquired chronic pure red cell aplasia (PRCA) following immunosuppressive therapy: a final report of the nationwide cohort study in 2004/2006 by the Japan PRCA collaborative study group. Br J Haematol. 2015;169:879–86.CrossRefPubMedGoogle Scholar