小児期に見られるmyelodysplastic syndrome(MDS)の臨床病態

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  • The Clinical Features of Childhood Myelodysplastic Syndrome(MDS).

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MDS in children is less common and has less clearcut findings than in adults. To clarify clinical features of childhood MDS, questionnaries were sent to all centers which had the members of the Japanese Society of Pediatric Hematology and the Japanese Society of Clinical Hematology. Data were collected on 165 patients treated between 1981 and 1990. The total group of patients consisted of 87 boys and 78 girls, aged 0-17 years. The distribution among the 5 subgroups of MDS was : RA 69, RARS 4, RAEB 36, CMMoL 13, RAEBT 37 and 6 patients who were difficult to classify. About 30% of patients exhibited bone marrow hypoplasia. Cytogenetic studies in 138 children showed karyotype abnormalities in 75; 35 and 13 had monosomy 7 and trisomy 8, respectively. Because of the generally poor prognosis, a prospective study of childhood MDS in Japan should be considered.

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