作者: Kamran Alimoghaddam , Masoud Iravani , Ardeshir Ghavamzadeh , Amir Ali Hamidieh , Arash Jalali
DOI:
关键词: Medicine 、 Cyclophosphamide 、 Internal medicine 、 Disease 、 Methotrexate 、 Regimen 、 Neutrophil Engraftment 、 Platelet Engraftment 、 Surgery 、 Hematopoietic stem cell transplantation 、 Rare disease
摘要: Introduction: Severe acquired aplastic anemia (SAA) is a rare disease and matched related hematopoietic stem cell transplantation (HSCT) the treatment of choice especially in pediatric patients. Immunosuppressive therapy alternative patients who do not have donor. We retrospectively analyzed received allogeneic HSCT at our institution. Methods: Between 1991 2011, 190 from HLA-matched donors (182 siblings 8 other relatives). Median age was 20.5 years (range 1 to 50 years). The graft source peripheral blood cells majority Conditioning regimen consist with myeloablative containing cyclophosphamide or without antithymocyte globulin. For graft-versus-host (GvHD) prophylaxis, we used cyclosporine methotrexate standard doses. Results: median follow-up time 30 months 3 year overall survival free 82% 75% respectively. neutrophil engraftment platelet 12 day 15 Grade 4 aGvHD occurred 26 (23.7%) chronic GvHD 46 (29.1%) survived 100 days after HSCT. At report 82.1% were alive normal hematologic parameters. failure rate about 8%. most common cause death GvHD. Conclusions: However an available SAA immunosuppressive therapy, should be seriously considered as therapeutic option particularly if sibling donor available. outcome center consistent result previous studies.