TY - JOUR
T1 - Long-term outcome of patients with acquired chronic pure red cell aplasia (PRCA) following immunosuppressive therapy
T2 - A final report of the nationwide cohort study in 2004/2006 by the Japan PRCA collaborative study group
AU - PRCA Collaborative Study Group
AU - Hirokawa, Makoto
AU - Sawada, Kenichi
AU - Fujishima, Naohito
AU - Teramura, Masanao
AU - Bessho, Masami
AU - Dan, Kazuo
AU - Tsurumi, Hisashi
AU - Nakao, Shinji
AU - Urabe, Akio
AU - Fujisawa, Shin
AU - Yonemura, Yuji
AU - Kawano, Fumio
AU - Oshimi, Kazuo
AU - Sugimoto, Koichi
AU - Matsuda, Akira
AU - Karasawa, Masamitsu
AU - Arai, Ayako
AU - Komatsu, Norio
AU - Harigae, Hideo
AU - Omine, Mitsuhiro
AU - Ozawa, Keiya
AU - Kurokawa, Mineo
N1 - Publisher Copyright:
© 2015 John Wiley & Sons Ltd.
PY - 2015/6/1
Y1 - 2015/6/1
N2 - Summary: Immunosuppressive therapy has been employed as the initial treatment for acquired chronic pure red cell aplasia (PRCA), such as idiopathic, thymoma-associated, or large granular lymphocyte (LGL) leukaemia-associated PRCA, which is thought to be immune-mediated. To explore the overall long-term outcome following immunosuppression and to identify the risk factors for death in these disorders, we conducted nationwide surveys in Japan 2004 and 2006, and identified a total of 185 patients with acquired chronic PRCA, including 72 idiopathic, 41 thymoma-associated and 14 LGL leukaemia-associated cases of PRCA for whom data was available. The present study evaluated 127 patients with these three subsets of PRCA. The median overall survival has not yet been reached in idiopathic PRCA. The estimated median overall survival times in patients with thymoma-associated and LGL leukaemia-associated PRCA were 142·1 and 147·8 months, respectively. Twenty-two deaths were reported, and the response to induction therapy and relapse of anaemia were found to be associated with death. The major causes of death were infection in seven patients and organ failure in another seven patients. The results suggest that maintenance therapy and the management of infectious complications are crucial for improving the prognosis of chronic PRCA.
AB - Summary: Immunosuppressive therapy has been employed as the initial treatment for acquired chronic pure red cell aplasia (PRCA), such as idiopathic, thymoma-associated, or large granular lymphocyte (LGL) leukaemia-associated PRCA, which is thought to be immune-mediated. To explore the overall long-term outcome following immunosuppression and to identify the risk factors for death in these disorders, we conducted nationwide surveys in Japan 2004 and 2006, and identified a total of 185 patients with acquired chronic PRCA, including 72 idiopathic, 41 thymoma-associated and 14 LGL leukaemia-associated cases of PRCA for whom data was available. The present study evaluated 127 patients with these three subsets of PRCA. The median overall survival has not yet been reached in idiopathic PRCA. The estimated median overall survival times in patients with thymoma-associated and LGL leukaemia-associated PRCA were 142·1 and 147·8 months, respectively. Twenty-two deaths were reported, and the response to induction therapy and relapse of anaemia were found to be associated with death. The major causes of death were infection in seven patients and organ failure in another seven patients. The results suggest that maintenance therapy and the management of infectious complications are crucial for improving the prognosis of chronic PRCA.
KW - Idiopathic
KW - Immunosuppressive therapy
KW - Large granular lymphocyte leukaemia
KW - Pure red cell aplasia
KW - Thymoma
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U2 - 10.1111/bjh.13376
DO - 10.1111/bjh.13376
M3 - Article
C2 - 25807974
AN - SCOPUS:84930274878
VL - 169
SP - 879
EP - 886
JO - British Journal of Haematology
JF - British Journal of Haematology
SN - 0007-1048
IS - 6
ER -