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21三体新生儿短暂性骨髓增殖性疾病的免疫表型

Immunophenotype of a transient myeloproliferative disorder in a newborn with trisomy 21.

作者信息

Girodon F, Favre B, Couillaud G, Carli P M, Parmeland C, Maynadié M

机构信息

Hematology Laboratory, University Hospital, Dijon, France.

出版信息

Cytometry. 2000 Apr 15;42(2):118-22. doi: 10.1002/(sici)1097-0320(20000415)42:2<118::aid-cyto6>3.0.co;2-e.

DOI:10.1002/(sici)1097-0320(20000415)42:2<118::aid-cyto6>3.0.co;2-e
PMID:10797450
Abstract

Cytologic, immunologic, and cytogenetic studies were performed on the blast cells of a newborn with Down syndrome and transient myeloproliferative disease. This hematologic disorder is uncommon, and occurs primarily in infants with Down syndrome. This boy presented with a high white blood cell count and a high percentage of blast cells, without anemia or thrombocytopenia. Chromosome analysis showed a constitutional trisomy 21 without any other clonal abnormality. A three-color flow cytometric analysis was performed and revealed two different CD45 dim, CD34(+), CD117(+), CD56(+) immature subpopulations: the normal immature myeloid precursor and an immature blast cell population that expressed CD41, CD42, CD61, CD36, CD13, CD1a, and CD2. We postulate that this population could be the leukemic precursor involved in the acute megakaryoblastic leukemia frequently observed in children with Down syndrome.

摘要

对一名患有唐氏综合征和短暂性骨髓增殖性疾病的新生儿的原始细胞进行了细胞学、免疫学和细胞遗传学研究。这种血液系统疾病并不常见,主要发生在唐氏综合征婴儿中。这个男孩表现为白细胞计数高和原始细胞百分比高,无贫血或血小板减少。染色体分析显示为先天性21三体,无任何其他克隆性异常。进行了三色流式细胞术分析,发现了两种不同的CD45暗淡、CD34(+)、CD117(+)、CD56(+)未成熟亚群:正常未成熟髓系前体和表达CD41、CD42、CD61、CD36、CD13、CD1a和CD2的未成熟原始细胞群体。我们推测这个群体可能是唐氏综合征患儿中常见的急性巨核细胞白血病所涉及的白血病前体。

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