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ADULT PATIENTS WITH ACQUIRED PURE RED CELL APLASIA: TREATED BY CYCLOSPORINE A OR CORTICOSTEROIDS：SIMILAR EFFICIENCY
Author(s):
Xuemei Wu
,
Xuemei Wu
Affiliations:
Hematological Department,The First Affiliate Hospital, Nanjing Medical University, Jiangsu Province Hospital,Nanjing,China
Suli Wang
,
Suli Wang
Affiliations:
Hematological Department,The First Affiliate Hospital, Nanjing Medical University, Jiangsu Province Hospital,Nanjing,China
Wenyi Shen
,
Wenyi Shen
Affiliations:
Hematological Department,The First Affiliate Hospital, Nanjing Medical University, Jiangsu Province Hospital,Nanjing,China
Guangsheng He
,
Guangsheng He
Affiliations:
Hematological Department,The First Affiliate Hospital, Nanjing Medical University, Jiangsu Province Hospital,Nanjing,China
Jianyong Li
Jianyong Li
Affiliations:
Hematological Department,The First Affiliate Hospital, Nanjing Medical University, Jiangsu Province Hospital,Nanjing,China
(Abstract release date: 05/18/17) EHA Library. He G. 05/18/17; 182466; PB1752
Dr. Guangsheng He
Dr. Guangsheng He
Contributions
PDF Abstract
Abstract

Abstract: PB1752

Type: Publication Only

Background
Adult pure red cell aplasia (PRCA) is a syndrome characterized by a severe normocytic anemia, reticulocytopenia, and absence of erythroblasts from an otherwise normal bone marrow. Immunosuppressive therapy has been used as the initial treatment foracquired chronic PRCA.

Aims
This article aims to evaluate the efficacy of cyclosporine A, and/or corticosteroids, and possible factors influencing it.

Methods
34 cases of PRCA were retrospectively analyzed at our institution. Clinical data of 23 inpatient cases and 11 outpatient cases since 2009 October were collected. These patients were treated by cyclosporine A (CsA), and/or corticosteroids (CS), or other immunosuppressive agents if become refractory and relapsed.

Results

31 patients were evaluated in our institution (one patient lost to follow-up and two patients with short observation period). The remission induction therapy included CsA (n=13), CS (n=13), or a simultaneous combination of CsA and CS (n=5). The initial response rate of CsA alone, CS alone, combination of CS and CsA were 69.2%, 46.2%, 80%, respectively (P=0.422). There was no statistical difference in response rate and CR rate between CsA-containing group and CS group, although the patients treated with CsA had a better response than those treated with CS (response rate 72.2%vs 46.2%, P=0.262; CR rate 33.3% vs 23.1%, P=0.696). Including patients who had crossed over from other treatment groups, the cumulative response rate of CsA, CS, combination of CS and CsA, was 73.7% (14/19), 46.7% (7/15), 83.3% (5/6), respectively (P=0.193); the cumulative rate of CR was 26.3% (5/19), 26.67% (4/15), 66.7% (4/6), respectively (P=0.202). In 23 refractory and relapsed PRCA patients, 8 out of 12 (66.7%) refractory patients and 4 out of 11 (36.4%) relapsed patients achieved remission. The response rate of treatment with traditional immunosuppressive agents (CS and/or CsA) was higher than other immunosuppressive agents (65.0% vs 20%, P=0.014).

Conclusion

CsA and/or CS are effective similarly in treating PRCA. For patients with relapse or refractory PRCA, there were no satisfactory treatment measures if CsA and/or CS were not be administered or un-effective. It was still needed to explore a more effective therapy for them.

Session topic: 12. Bone marrow failure syndromes incl. PNH - Clinical

Keyword(s): Pure red cell aplasia

Abstract: PB1752

Type: Publication Only

Background
Adult pure red cell aplasia (PRCA) is a syndrome characterized by a severe normocytic anemia, reticulocytopenia, and absence of erythroblasts from an otherwise normal bone marrow. Immunosuppressive therapy has been used as the initial treatment foracquired chronic PRCA.

Aims
This article aims to evaluate the efficacy of cyclosporine A, and/or corticosteroids, and possible factors influencing it.

Methods
34 cases of PRCA were retrospectively analyzed at our institution. Clinical data of 23 inpatient cases and 11 outpatient cases since 2009 October were collected. These patients were treated by cyclosporine A (CsA), and/or corticosteroids (CS), or other immunosuppressive agents if become refractory and relapsed.

Results

31 patients were evaluated in our institution (one patient lost to follow-up and two patients with short observation period). The remission induction therapy included CsA (n=13), CS (n=13), or a simultaneous combination of CsA and CS (n=5). The initial response rate of CsA alone, CS alone, combination of CS and CsA were 69.2%, 46.2%, 80%, respectively (P=0.422). There was no statistical difference in response rate and CR rate between CsA-containing group and CS group, although the patients treated with CsA had a better response than those treated with CS (response rate 72.2%vs 46.2%, P=0.262; CR rate 33.3% vs 23.1%, P=0.696). Including patients who had crossed over from other treatment groups, the cumulative response rate of CsA, CS, combination of CS and CsA, was 73.7% (14/19), 46.7% (7/15), 83.3% (5/6), respectively (P=0.193); the cumulative rate of CR was 26.3% (5/19), 26.67% (4/15), 66.7% (4/6), respectively (P=0.202). In 23 refractory and relapsed PRCA patients, 8 out of 12 (66.7%) refractory patients and 4 out of 11 (36.4%) relapsed patients achieved remission. The response rate of treatment with traditional immunosuppressive agents (CS and/or CsA) was higher than other immunosuppressive agents (65.0% vs 20%, P=0.014).

Conclusion

CsA and/or CS are effective similarly in treating PRCA. For patients with relapse or refractory PRCA, there were no satisfactory treatment measures if CsA and/or CS were not be administered or un-effective. It was still needed to explore a more effective therapy for them.

Session topic: 12. Bone marrow failure syndromes incl. PNH - Clinical

Keyword(s): Pure red cell aplasia

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