Japan National Registry of Aplastic Anemia in Children 1988-2000: Clinical Features and Prognosis of Idiopathic Aplastic Anemia
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概要
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The aplastic anemia committee has undertaken annual nationwide survey of children with aplastic anemia (AA). As of 2002, 760 idiopathic AA patients (diagnosis year 1988-2000) have been registered and followed up. Numbers of newly diagnosed patients in each year ranged from 45 to 82. Severe AA patients accounted for 53% of these patients. Over 80% of patients who were diagnosed after 1994 underwent bone marrow cell chromosomes analysis, and 9 of 336 patients showed abnormalities at their presentation. The survival rates of very severe and severe patients who were diagnosed after 1994 improved (Kaplan-Meier [KM] survival 86.7 ± 5.3%, 87.5 ± 3.8%); however that of moderate and mild patients did not. It should be of concern that some of the mild patients died over 5 years later. Prognosis of allogeneic bone marrow transplantation from HLA matched siblings was good. If patients received HLA matched unrelated bone marrow transplantation within 2 years, the prognosis was fair (KM survival 86.4 ± 6.3%). The number of MDS/ leukemia transfbrmation decreased after 1994. This annual nationwide survey is expected to provide useful information for every clinician and patient.
- 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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