Published online 18 July 2008
Haematologica, Vol 93, Issue 10, 1555-1559 doi:10.3324/haematol.12871
Copyright © 2008 by Ferrata Storti Foundation
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Disorders of Erythropoiesis

Long-term responses and outcomes following immunosuppressive therapy in large granular lymphocyte leukemia-associated pure red cell aplasia: a Nationwide Cohort Study in Japan for the PRCA Collaborative Study Group

Naohito Fujishima1, Ken-ichi Sawada1, Makoto Hirokawa1, Kazuo Oshimi2, Koichi Sugimoto2, Akira Matsuda3, Masanao Teramura4, Masamitsu Karasawa5, Ayako Arai6, Yuji Yonemura7, Shinji Nakao8, Akio Urabe9, Mitsuhiro Omine10, Keiya Ozawa11

1 Division of Hematology and Oncology, Department of Medicine, Akita University School of Medicine, Akita, Akita
2 Division of Hematology, Juntendo University School of Medicine, Bunkyo-ku, Tokyo
3 Department of Hematology, Saitama International Medical Center, Saitama Medical University, Hidaka, Saitama
4 Department of Hematology, Tokyo Women’s Medical University, Shinjuku-ku, Tokyo
5 Blood Transfusion Service, Gunma University Hospital, Maebashi, Gunma
6 Department of Hematology, Tokyo Medical and Dental University, Bunkyo-ku, Tokyo
7 Blood Transfusion Service, Kumamoto University School of Medicine, Kumamoto, Kumamoto
8 Cellular Transplantation Biology, Kanazawa University Graduate School of Medical Science, Kanazawa, Ishikawa
9 Division of Hematology, NTT Kanto Medical Center, Shinagawa, Tokyo
10 Internal Medicine, Showa University Fujigaoka Hospital, Yokohama, Kanagawa
11 Division of Hematology, Department of Medicine, Jichi Medical University, Shimotsuke, Tochigi, Japan

Correspondence: Naohito Fujishima, M.D., Division of Hematology and Oncology, Department of Medicine, Akita University School of Medicine, 1-1-1 Hondo, Akita 010-8543, Japan. E-mail:naofuji{at}doc.med.akita-u.ac.jp

Large granular lymphocyte leukemia-associated pure red cell aplasia accounts for a significant portion of secondary pure red cell aplasia cases. However, because of its rarity, long-term responses and relapse rates after immunosuppressive therapy are largely unknown. We conducted a nationwide survey in Japan and collected 185 evaluable patients. Fourteen patients with large granular lymphocyte leukemia-associated pure red cell aplasia were evaluated. Cyclophosphamide, cyclosporine A and prednisolone produced remissions in 6/8, 1/4 and 0/2 patients respectively. Seven and 5 patients were maintained on cyclophosphamide or cyclosporine A respectively. Two patients relapsed after stopping cyclophosphamide, and 2 patients relapsed during maintenance therapy with cyclosporine A. The median relapse-free survival in the cyclophosphamide - and the cyclosporine A groups was 53 and 123 months respectively. Large granular lymphocyte leukemia-associated pure red cell aplasia showed a good response to either cyclophosphamide or cyclosporine A. Most patients continued to receive maintenance therapy and it remains uncertain whether cyclophosphamide or cyclosporine A can induce a maintenance-free hematologic response in large granular lymphocyte leukemia-associated pure red cell aplasia.

Key words: pure red cell aplasia, large granular lymphocyte leukemia, cyclophosphamide, cyclosporine.




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R. Zambello and G. Semenzato
Large granular lymphocyte disorders: new etiopathogenetic clues as a rationale for innovative therapeutic approaches
Haematologica, October 1, 2009; 94(10): 1341 - 1345.
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