MYELODYSPLASIA AND ACUTE MEGAKARYOBLASTIC LEUKEMIA IN DOWNS-SYNDROME

被引:120
作者
ZIPURSKY, A
THORNER, P
DEHARVEN, E
CHRISTENSEN, H
DOYLE, J
机构
[1] UNIV TORONTO,DEPT PATHOL,TORONTO M5S 1A1,ONTARIO,CANADA
[2] HOSP SICK CHILDREN,DEPT PEDIAT,DIV HEMATOL ONCOL,TORONTO M5G 1X8,ONTARIO,CANADA
[3] HOSP SICK CHILDREN,DEPT PATHOL,TORONTO M5G 1X8,ONTARIO,CANADA
关键词
MEGAKARYOBLASTS; MEGAKARYOBLASTIC LEUKEMIA; MYELODYSPLASIA; DOWNS SYNDROME;
D O I
10.1016/0145-2126(94)90111-2
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
In this report we describe the clinical and hematologic features of 23 cases of myelodysplasia (MDS) or acute megakaryoblastic leukemia (AMKL) occurring in Down's syndrome. MDS was characterized by thrombocytopenia, abnormal megakaryocytopoiesis, megakaryoblasts (<30%) in the marrow and abnormal karyotype, the most common of which was trisomy 8, found in 7/15 patients with MDS. Three of five patients achieved a complete remission with low dose cytosine arabinoside, vincristine and retinyl palmitate. The high cure rate and the distinctive features of the leukemic process in these cases suggest that this type of MDS and AMKL are unique to patients with Down's syndrome.
引用
收藏
页码:163 / 171
页数:9
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