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
Authors:
- Ken-Ichi SawadaKSKen-Ichi Sawada
- Makoto HirokawaMHMakoto Hirokawa
- Naohito FujishimaNFNaohito Fujishima
- Masanao TeramuraMTMasanao Teramura
- Masami BesshoMBMasami Bessho
- Kazuo DanKDKazuo Dan
- Hisashi TsurumiHTHisashi Tsurumi
- Shinji NakaoSNShinji Nakao
- Akio UrabeAUAkio Urabe
- Mitsuhiro OmineMOMitsuhiro Omine
- Keiya OzawaKOKeiya Ozawa
- PRCA Collaborative Study GroupPGPRCA Collaborative Study Group
- 1 August 2007
- journal article
- Published by Ferrata Storti Foundation (Haematologica) in Haematologica
- Vol. 92 (8) , 1021-1028
Abstract
Background and Objectives Cyclosporine A (CsA) has become one of the leading agents for the treatment of pure red cell aplasia (PRCA). However, further studies are necessary to determine the relapse-free survival (RFS) and overall survival (OS) of patients treated with this drug, the minimum duration of therapy for induction of remission, and whether or not there is need for maintenance treatment. Design and Methods We conducted a nationwide survey in Japan. From a total of 185 patients (with 73 primary idiopathic PRCA and 112 with secondary PRCA), we evaluated 62 patients with primary idiopathic PRCA for this report. Results The remission induction therapy for these patients included CsA (n=31), corticosteroids (CS) (n=20) or other drugs (n=11). CsA and CS produced remissions in 23 (74%) and 12 (60%) patients, respectively. The salvage treatment produced remissions in 58 patients (94%). Forty-one and 15 patients were maintained on CsA±CS (CsA-containing group) or CS alone (CS group), respectively. The median RFS in the CsA-containing group was 103 months, longer than that seen in the CS group (33 months) (ppp=0.104). Interpretation and Conclusions CsA-containing regimens sustain prolonged RFS more effectively than CS in primary idiopathic PRCA and seem to be important to prevent relapse.Keywords
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