Differentiation of blast cells from a Down's syndrome patient with transient myeloproliferative disorder.
Differentiation of blast cells from a Down's syndrome patient with transient myeloproliferative disorder.
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来自患有短暂性骨髓增殖性疾病的唐氏综合症患者的母细胞的分化。
DOI：
10.1182/blood.v69.2.508.508
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发表时间：
1987
期刊：
影响因子：
20.3
通讯作者：
M. Saito
中科院分区：
文献类型：
--
作者：
J. Suda;M. Eguchi;Y. Akiyama;Y. Iwama;T. Furukawa;Y. Sato;Y. Miura;T. Suda;M. Saito
本文报告一例先天性骨髓增生性疾病合并唐氏综合征的男性新生儿。他的血象显示白细胞增多与成熟细胞和大量原始细胞共存。用液体培养和甲基纤维素培养系统研究了植物血凝素刺激的白细胞条件培养液（PHA-LCM）对原始细胞体外增殖和分化的影响。通过特异性细胞化学染色、电子显微镜和免疫学研究证实了胚细胞分化为髓样细胞。患者血浆中无特异性因子促进母细胞增殖或分化。通过显微操作技术研究了单胚细胞在甲基纤维素培养物中生长的集落的细胞组成。观察到高的镀覆效率。在136个培养物中，78个显示菌落生长。半数原始细胞为集落形成细胞，在PHA-LCM存在下可增殖并分化为嗜碱性粒细胞、中性粒细胞、嗜酸性粒细胞、巨噬细胞和红细胞。利用向嗜碱性粒细胞途径分化能力强的原始细胞，研究了重组粒细胞-巨噬细胞集落刺激因子（GM-CSF）的作用。重组GM-CSF支持中性粒细胞、嗜酸性粒细胞和巨噬细胞，但不支持典型的嗜碱性粒细胞。胚细胞在体外分化为各种细胞的这些发现与该患者的嗜酸性粒细胞增多症、嗜酸性粒细胞增多症、嗜碱性粒细胞增多症和血小板增多症的发现一致。
A male neonate with Down's syndrome and congenital myeloproliferative disorder was studied. His blood picture showed the unique coexistence of leukocytosis with matured cells and a large number of blast cells. The in vitro proliferation and differentiation of blast cells into various lineages in the presence of phytohemagglutinin-stimulated leukocyte conditioned medium (PHA-LCM) was examined by using a liquid culture and a methylcellulose culture system. The differentiation of blast cells into myeloid cells was confirmed by specific cytochemical stainings, electron microscopy, and an immunologic study. No specific factors in the plasma of the patient promoted the proliferation or differentiation of blast cells. The cellular composition of colonies grown in methylcellulose culture from single blast cells was studied by a micromanipulation technique. High plating efficiency was observed. Of 136 cultures, 78 showed colony growth. Half of the blast cells were colony-forming cells that could proliferate and differentiate into basophils, neutrophils, eosinophils, macrophages, and erythrocytes in the presence of PHA-LCM. Using the blast cells with a high differentiation capacity to the basophil pathway, we studied the effect of recombinant granulocyte-macrophage colony-stimulating factor (GM-CSF). Recombinant GM-CSF support neutrophils, eosinophils, and macrophages but not typical basophils. These findings of the cell differentiation of blast cells into various kinds of cells in vitro were in agreement with the finding of neutrophilia, eosinophilia, basophilia, and thrombocythemia in this patient.
患有唐氏综合症的白血病：短暂性先天性骨髓增殖综合症后急性粒单核细胞白血病中 1 号和 19 号染色体之间的易位。
影响因子：
20.3
作者：
Morgan,R;Hecht,F;Cleary,ML;Sklar,J;Link,MP
通讯作者：
Link,MP