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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
Author(s) -
Hirokawa Makoto,
Sawada Kenichi,
Fujishima Naohito,
Teramura Masanao,
Bessho Masami,
Dan Kazuo,
Tsurumi Hisashi,
Nakao Shinji,
Urabe Akio,
Fujisawa Shin,
Yonemura Yuji,
Kawano Fumio,
Oshimi Kazuo,
Sugimoto Koichi,
Matsuda Akira,
Karasawa Masamitsu,
Arai Ayako,
Komatsu Norio,
Harigae Hideo,
Omine Mitsuhiro,
Ozawa Keiya,
Kurokawa Mineo
Publication year - 2015
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.13376
Subject(s) - medicine , pure red cell aplasia , thymoma , immunosuppression , cohort , aplasia , immunology , pediatrics , anemia
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. Thepresent 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 .