Diamond-Blackfan Anemia in Japan: The Epidemiology and Treatment Response:The Epidemiology and Treatment Response
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概要
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The epidemiology and treatment responses of Diamond-Blackfan anemia (DBA) were surveyed, based on the cohort of patients < 16years of age registered by the Japanese Society of Pediatric Hematology from 1988to 1998. The registered number was 54 (M : F=26 : 28), and the annual incidence was calculated to be 4.02 cases/million births. The median age at diagnosis was 60 days, and 59% presented within 3 months of age. Three patients had a familial occurrence. All patients received prednisolone (PSL). Cyclosporine A (CsA) was combined in 17 patients. Forty-seven received transfusions, and 13 underwent hematopoietic stem cell transplantation (HSCT). The cumulative probability of a medication- or transfusion-free state prior to HSCT was 36% or 69% > 5 years after diagnosis. Thirteen patients were weaned from PSL-therapy without HSCT. Adjunct CsA was not associated with weaning from the therapy. Transfusion and medication were stopped at 249 and 933 days after diagnosis, respectively, in 13 patients who achieved a state of independency. No initial findings could predict the treatment dependency. More than 20% of patients sustained hemosiderosis and/or adverse effects of PSL. The ages and reticulocyte counts at diagnosis in patients who underwent HSCT were lower than in those who did not. HSCT lead to the highest success (85%) of all previous reports, despite the fact that 5 alternative donors were included in our results. Two unrelated cord blood transplantations failed. These results suggest the need for developing an integral treatment strategy including selective HSCT for the patients with refractory DBA.
- 特定非営利活動法人 日本小児血液・がん学会の論文
特定非営利活動法人 日本小児血液・がん学会 | 論文
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