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著者: Naohito Fujishima, Ken-ichi Sawada, Makoto Hirokawa, Kazuo Oshimi, Koichi Sugimoto, Akira Matsuda, Masanao Teramura, Masamitsu Karasawa, Ayako Arai, Yuji Yonemura, Shinji Nakao, Akio Urabe, Mitsuhiro Omine, Keiya Ozawa, PRCA Collaborative Study Group
雑誌名: Haematologica. 2008 Oct;93(10):1555-9. doi: 10.3324/haematol.12871. Epub 2008 Jul 18.
Abstract/Text
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.
PMID 18641028 Haematologica. 2008 Oct;93(10):1555-9. doi: 10.3324/haematol.12871. Epub 2008 Jul 18.
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