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

• Published in: Haematologica (Roma) 2008-10, Vol.93 (10), p.1555-1559
• Main Authors: Fujishima, Naohito, Sawada, Ken-ichi, Hirokawa, Makoto, Oshimi, Kazuo, Sugimoto, Koichi, Matsuda, Akira, Teramura, Masanao, Karasawa, Masamitsu, Arai, Ayako, Yonemura, Yuji, Nakao, Shinji, Urabe, Akio, Omine, Mitsuhiro, Ozawa, Keiya
• Format: Article
• Language: English
• Subjects: Adult; Aged; Aged, 80 and over; Biological and medical sciences; Cohort Studies; Cooperative Behavior; Female; Follow-Up Studies; Hematologic and hematopoietic diseases; Humans; Immunosuppressive Agents - pharmacology; Japan - epidemiology; Leukemia, Large Granular Lymphocytic - epidemiology; Leukemia, Large Granular Lymphocytic - etiology; Leukemia, Large Granular Lymphocytic - immunology; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Male; Medical sciences; Middle Aged; Red-Cell Aplasia, Pure - complications; Red-Cell Aplasia, Pure - epidemiology; Red-Cell Aplasia, Pure - immunology; Remission Induction; Salvage Therapy; Survival Rate; Time Factors; Treatment Outcome
• ISSN: 0390-6078; 1592-8721
• DOI: 10.3324/haematol.12871

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. K