Leukemia in Down syndrome: a review.
Leukemia in Down syndrome: a review.
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唐氏综合症中的白血病：综述。
DOI：
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发表时间：
1992
影响因子：
1.7
通讯作者：
J. Doyle
中科院分区：
文献类型：
--
作者：
A. Zipursky;Annete Poon;J. Doyle
唐氏综合征（Down syndrome，DS）患儿白血病的发病率高于正常儿童。在大约50%的情况下，白血病的类型是急性巨核细胞白血病（AMKL），并且它发生在生命的前4年。白血病细胞还具有红系祖细胞的特征，因此似乎是具有双表型特性的前体细胞。此外，患有DS的新生儿经常发生短暂性白血病（TL），其特征是血液中存在巨核细胞，这些细胞在生命的前1-3个月内消失。这种疾病的发病率是未知的，虽然初步研究表明，巨核母细胞可能经常在DS新生儿的血液中发现。TL不会发生在正常新生儿中。虽然TL会自发消失，但这些儿童中的许多人会在1-4岁时发展为AMKL。最近的调查表明，20-30%的TL新生儿将发展AMKL。初步证据表明，TL是一种克隆性增殖，可能是致命的，可能发生在DS儿童的特定亚组中。本报告中的观察结果来自我们自己的经验、文献报告和加拿大唐氏综合征白血病登记处积累的数据。
The incidence of leukemia is higher in children with Down syndrome (DS) than in normals. In approximately 50% of cases the type of leukemia is acute megakaryoblastic leukemia (AMKL) and it occurs during the first 4 years of life. The leukemic cell also has features of erythroid progenitors and therefore appears to be a precursor cell with biphenotypic properties. In addition, newborns with DS frequently develop transient leukemia (TL), which is characterized by the presence of megakaryoblasts in the blood which disappear during the first 1-3 months of life. The incidence of this disorder is unknown although preliminary studies suggest that megakaryoblasts may be found frequently in the blood of DS newborns. TL does not occur in normal newborn infants. Although TL disappears spontaneously, many of these children will develop AMKL at 1-4 years of age. Recent surveys suggest that 20-30% of newborns with TL will develop AMKL. Preliminary evidence suggests that TL is a clonal proliferation, can be fatal, and may occur in a specific subgroup of DS children. The observations in this report are drawn from our own experience, reports in the literature, and data accumulated in the Canadian Down Syndrome Leukemia Registry.
影响因子：
158.5
作者：
ANTONARAKIS, SE
通讯作者：
ANTONARAKIS, SE