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真性红细胞增多证是骨髓增生异常疾患之一。骨髓中以红系增生为主,并常常伴有粒系、巨核系的增多。因此,它可能是一种造血干细胞疾患。在正常生理情况下,缺氧可以促使体内红细胞生成素(Erythropoietim简称Epo)水平增高,从而导致反应性的红系造血的增加。真红体内并无Epo水平增高。因此其红细胞增加是一种异常的骨髓增生现象。 70年代初应用体外培养红系祖细胞技术发现真红体内除了存在着对Epo正常反应的红系祖细胞外,还存在着另一不依赖Epo调节的可自我增殖的异常红系祖细胞群体。以后应用G-6-PD同功酶技术进一步地证实了这个异常细胞群体的存在。并证实这
The syndrome of polycythemia vera is one of the myelodysplastic disorders. In the bone marrow, erythroid hyperplasia is predominant and often accompanied by an increase in granular and megakaryocytes. Therefore, it may be a hematopoietic stem cell disorder. Under normal physiological conditions, hypoxia can increase the level of erythropoietin (Epo) in the body, leading to an increase in reactive erythroid hematopoiesis. There is no increase in Epo levels in the true red body. Therefore, its increase in red blood cells is an abnormal phenomenon of bone marrow hyperplasia. In the early 1970s, the use of in vitro cultured erythroid progenitor cells revealed that in addition to erythroid progenitors that normally respond to Epo in the true red body, there is another population of abnormal erythroid progenitor cells that is independent of Epo regulation and can self-regenerate. The use of G-6-PD isoenzyme technology later confirmed the presence of this abnormal cell population. And confirm this