title: 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.
abstract: 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.
authors.affiliation: Division of Hematology and Oncology, Department of Medicine, Akita University School of Medicine, 1-1-1 Hondo, Akita 010-8543, Japan. naofuji@doc.med.akita-u.ac.jp
authors.forename: Naohito
authors.initials: N
authors.lastname: Fujishima
authors.forename: Ken-ichi
authors.initials: K
authors.lastname: Sawada
authors.forename: Makoto
authors.initials: M
authors.lastname: Hirokawa
authors.forename: Kazuo
authors.initials: K
authors.lastname: Oshimi
authors.forename: Koichi
authors.initials: K
authors.lastname: Sugimoto
authors.forename: Akira
authors.initials: A
authors.lastname: Matsuda
authors.forename: Masanao
authors.initials: M
authors.lastname: Teramura
authors.forename: Masamitsu
authors.initials: M
authors.lastname: Karasawa
authors.forename: Ayako
authors.initials: A
authors.lastname: Arai
authors.forename: Yuji
authors.initials: Y
authors.lastname: Yonemura
authors.forename: Shinji
authors.initials: S
authors.lastname: Nakao
authors.forename: Akio
authors.initials: A
authors.lastname: Urabe
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Omine
authors.forename: Keiya
authors.initials: K
authors.lastname: Ozawa
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015331:Cohort Studies; D003299:Cooperative Behavior; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents / Q000494:pharmacology*; D007564:Japan / Q000453:epidemiology; D054066:Leukemia, Large Granular Lymphocytic / Q000453:epidemiology* / Q000209:etiology / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D012010:Red-Cell Aplasia, Pure / Q000150:complications / Q000453:epidemiology* / Q000276:immunology*; D012074:Remission Induction; D016879:Salvage Therapy; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents