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原始造血干细胞在骨髓增生异常综合征中呈现多克隆模式。

Primitive hematopoietic stem cells shows a polyclonal pattern in myelodysplastic syndromes.

作者信息

Guidetti Francesca, Grazioli Sandra, Capelli Francesca, Marini Claudia, Gallicchio Margherita, De Micheli Daniela, Castello Luigi, Sainaghi Pier Paolo, Fra Gian Paolo, Saglio Giuseppe, Avanzi Gian Carlo

机构信息

Department of Medical Science, University of Piemonte Orientale Amedeo Avogadro Novara, Italy.

出版信息

Haematologica. 2004 Jan;89(1):21-8.

Abstract

BACKGROUND AND OBJECTIVES

Clonal hematopoiesis is the hallmark of myelodysplastic syndromes, but the role played by pluripotent stem cells and progenitor cells in these disorders remains unclear.

DESIGN AND METHODS

Eight female patients with myelodysplastic syndrome were studied. X-chromosome inactivation patterns were analyzed in peripheral blood granulocytes, T-lymphocytes, single colonies originating from bone marrow progenitors and pluripotent stem cells, using the human androgen receptor locus polymorphism assay.

RESULTS

Granulocytes and progenitor cells were monoclonal in 7/8 cases. Immature stem cells showed a non-clonal pattern of X-inactivation and were detectable at diagnosis in the presence of clonal hematopoiesis. T-lymphocyte clonality was heterogeneous.

INTERPRETATION AND CONCLUSIONS

In myelodysplastic syndromes, hematopoiesis may be dominated by a neoplastic clone by virtue of its biological advantage over a residual polyclonal, probably still normal, population of immature stem cells still able to grow in vitro.

摘要

背景与目的

克隆性造血是骨髓增生异常综合征的标志,但多能干细胞和祖细胞在这些疾病中所起的作用仍不清楚。

设计与方法

对8例女性骨髓增生异常综合征患者进行了研究。采用人类雄激素受体基因座多态性分析方法,对外周血粒细胞、T淋巴细胞、源自骨髓祖细胞和多能干细胞的单个集落进行X染色体失活模式分析。

结果

8例中有7例的粒细胞和祖细胞为单克隆性。未成熟干细胞显示出非克隆性的X失活模式,且在诊断时克隆性造血存在的情况下可检测到。T淋巴细胞的克隆性是异质性的。

解读与结论

在骨髓增生异常综合征中,造血可能由一个肿瘤性克隆主导,因为它相对于残余的多克隆、可能仍正常的未成熟干细胞群体具有生物学优势,这些未成熟干细胞仍能在体外生长。

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