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Journal of the Korean Pediatric Society 1993;36(1):9-16.
Acute Megakaryoblastic Leukemia in Children
Hong Hoe Koo, Sang Kyu Park, Eun Sil Dong, Hye Jung Park, Hee Young Shin, Hyo Seop Ahn
Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea
급성 거핵아세포성 백혈벙에 관한 임상적 고찰
구홍회, 박상규, 동은실, 박혜정, 신희영, 안효섭
서울대학교 의과대학 소아과학교실
Abstract
We analyzed the clinical and laboratory features of ten children with acute megakaryoblastic leukemia (M7) and compared the findings with those reported in the literature. The diagnosis was supported by ultrastructural examination of platelet peroxidase or immunophenotyping for glycoprotein Iib/IIIa. Of the ten children, five were girls and five were boys. The median age at diagnosis was 13 months. Two patients had prominent myelofibrosis and one patient had Down syndrome. Nine was 13 months. Two patients had prominent myelofibrosis and one patient had Down syndrome. Nine patients were treated with low-dose cytosine arabinoside (10mg/m2) administered intravenously, or subcutaneously, or intramuscularyly, twice daily in 21 day courses. Seven patients achieved hematologic response and three patients are alive without evidence of disease. The 4 year event free survival rate was 30.0%. It is our impression that the prevalence of acute megakaryoblastic leukemia has been under-estimated, and low-dose cytosine arabinoside treatment may be of value in its management. This approach may be particularily useful in hospitals with scarce well-equipped facilities, since this protocol does not induce profound marrow hypopasia and intensive supportive measures are not required as they would be with the use of more aggressive drug combination.
Key Words: Acute megakaryobastic leukemia, Children, Low-dose Ara-C


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