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慢性髓性白血病中的造血干细胞。

Hematopoietic stem cells in chronic myeloid leukemia.

作者信息

Petzer Andreas L, Gunsilius Eberhard

机构信息

Stem Cell Laboratory, Division of Hematology & Oncology, University Hospital Innsbruck, Innsbruck, Austria.

出版信息

Arch Med Res. 2003 Nov-Dec;34(6):496-506. doi: 10.1016/j.arcmed.2003.09.005.

Abstract

Chronic myeloid leukemia (CML) is a malignant myeloproliferative disorder originating from a pluripotent hematopoietic stem cell that acquires a Philadelphia (Ph) chromosome encoding the BCR-ABL oncogenic fusion protein. This molecular abnormality that is thought to be causative in CML was the first acquired chromosome translocation associated with a human malignancy. This chromosomal translocation also makes it possible to precisely distinguish between residual normal (i.e., Ph-, BCR-ABL-) progenitor or stem cells and their leukemic counterpart, Ph+ or BCR-ABL+ progenitor/stem cells in every given sample of a patient with CML. This has provided seminal insights into the molecular and cellular biology of leukemia and also of the process of normal hematopoiesis. CML has become a fascinating model disease for malignancy in general.

摘要

慢性粒细胞白血病(CML)是一种恶性骨髓增殖性疾病,起源于多能造血干细胞,该干细胞获得了编码BCR-ABL致癌融合蛋白的费城(Ph)染色体。这种被认为是CML致病原因的分子异常是首例与人类恶性肿瘤相关的获得性染色体易位。这种染色体易位还使得在慢性粒细胞白血病患者的每个给定样本中,能够精确区分残留的正常(即Ph-、BCR-ABL-)祖细胞或干细胞与其白血病对应物,即Ph+或BCR-ABL+祖细胞/干细胞。这为白血病以及正常造血过程的分子和细胞生物学提供了开创性的见解。总体而言,慢性粒细胞白血病已成为一种引人入胜的恶性肿瘤模型疾病。

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