Acute megakaryoblastic leukemia in children.
- Author:
Hong Hoe KOO
;
Eun Sil DONG
;
Hye Jung PARK
;
Hee Young SHIN
;
Hyo Seop AHN
;
Sang Kyu PARK
- Publication Type:Original Article
- Keywords:
Acute megakaryoblastic leukemia;
Children;
Low-dose Ara-C
- MeSH:
Blood Platelets;
Bone Marrow;
Child*;
Cytarabine;
Diagnosis;
Disease-Free Survival;
Down Syndrome;
Female;
Glycoproteins;
Humans;
Immunophenotyping;
Leukemia, Megakaryoblastic, Acute*;
Megakaryocyte Progenitor Cells;
Peroxidase;
Prevalence;
Primary Myelofibrosis
- From:Journal of the Korean Pediatric Society
1993;36(1):9-16
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
We analyzed the clinical and laboratory features of ten children with acute megakaryoblastic lukemia (M7)and compared the findings with those reported in the literature. The diagnosis was supprted 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 patients were treatd with low-dose cytosine arabinoside (10mg/m2)administered intravenously, or subcutaneously, or intramuscularly, 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 was30.0%. 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 hypoplasia and intensive supportive measures are not required as they would be with the use of more aggressive drug combination.
