title: 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.
abstract: 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.
authors: Department of General Internal Medicine and Clinical Laboratory Medicine, Akita University Graduate School of Medicine, Akita, Japan.
authors: Makoto
authors: M
authors: Hirokawa
authors: Department of Haematology, Nephrology and Rheumatology, Akita University Graduate School of Medicine, Akita, Japan.
authors: Kenichi
authors: K
authors: Sawada
authors: Department of Haematology, Nephrology and Rheumatology, Akita University Graduate School of Medicine, Akita, Japan.
authors: Naohito
authors: N
authors: Fujishima
authors: Department of Haematology, Tokyo Women's Medical University, Tokyo, Japan.
authors: Masanao
authors: M
authors: Teramura
authors: Haematology Division, Department of Internal Medicine, Saitama Medical University, Saitama, Japan.
authors: Masami
authors: M
authors: Bessho
authors: Department of Haematology, Nippon Medical School, Tokyo, Japan.
authors: Kazuo
authors: K
authors: Dan
authors: First Department of Internal Medicine, Gifu University School of Medicine, Gifu, Japan.
authors: Hisashi
authors: H
authors: Tsurumi
authors: Department of Cellular Transplantation Biology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan.
authors: Shinji
authors: S
authors: Nakao
authors: Division of Haematology, NTT Medical Centre, Tokyo, Japan.
authors: Akio
authors: A
authors: Urabe
authors: Department of Haematology, Yokohama City University Medical Centre, Yokohama, Kanagawa, Japan.
authors: Shin
authors: S
authors: Fujisawa
authors: Department of Transfusion Medicine and Cell Therapy Blood Transfusion Service, Kumamoto University School of Medicine, Kumamoto, Japan.
authors: Yuji
authors: Y
authors: Yonemura
authors: National Hospital Organization Kumamoto National Hospital Medical Centre, Kumamoto, Japan.
authors: Fumio
authors: F
authors: Kawano
authors: Division of Haematology, Juntendo University School of Medicine, Tokyo, Japan.
authors: Kazuo
authors: K
authors: Oshimi
authors: Division of Haematology, Juntendo University School of Medicine, Tokyo, Japan.
authors: Koichi
authors: K
authors: Sugimoto
authors: Saitama International Medical Centre, Saitama Medical University, Saitama, Japan.
authors: Akira
authors: A
authors: Matsuda
authors: Blood Transfusion Service, Gunma University Hospital, Maebashi, Gunma, Japan.
authors: Masamitsu
authors: M
authors: Karasawa
authors: Department of Haematology, Tokyo Medical and Dental University, Tokyo, Japan.
authors: Ayako
authors: A
authors: Arai
authors: Division of Haematology, Juntendo University School of Medicine, Tokyo, Japan.
authors: Norio
authors: N
authors: Komatsu
authors: Department of Haematology and Immunology, Tohoku University Graduate School of Medicine, Sendai, Japan.
authors: Hideo
authors: H
authors: Harigae
authors: Division of Haematology, Internal Medicine, Showa University Fujigaoka Hospital, Yokohama, Japan.
authors: Mitsuhiro
authors: M
authors: Omine
authors: Division of Haematology, Department of Medicine, Jichi Medical School, Tochigi, Japan.
authors: Keiya
authors: K
authors: Ozawa
authors: Department of Haematology and Oncology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
authors: Mineo
authors: M
authors: Kurokawa
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002423:Cause of Death; D002908:Chronic Disease; D015331:Cohort Studies; D015897:Comorbidity; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012008:Recurrence; D012010:Red-Cell Aplasia, Pure / Q000188:drug therapy* / Q000453:epidemiology / Q000209:etiology* / Q000401:mortality; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents
keywords: idiopathic; immunosuppressive therapy; large granular lymphocyte leukaemia; pure red cell aplasia; thymoma