Singer J W, Adamson J W, Ernst C, Lin N, Steinmann L, Murphy S, Fialkow P J
J Clin Invest. 1980 Oct;66(4):730-5. doi: 10.1172/JCI109910.
In previous studies of two patients with polycythemia vera (PV) who were heterozygous at the X-linked locus for glucose-6-phosphate dehydrogenase (G6PD), only A type enzyme was found in nonlymphoid blood cells. However, some erythroid and granulocytic colonies grown in vitro were type B and therefore arose from presumably normal progenitors. One patient had enough type B colonies (8%) that studies of the physical characteristics of normal and PV clonal colony-forming cells could be undertaken. When marrow cells were separated by velocity sedimentation at unit gravity, most PV clonal granulocyte-macrophage progenitors (CFU-C) (type A G6PD) sedimented between 6.4 and 7.2 mm/h, whereas most residual normal, type B CFU-C sedimented less than or equal to 5.9 mm/h (P = 0.04)., When blood cells were separated over a discontinuous buoyant density gradient, PV clonal CFU-C equilibrated at densities < 1.065 g/ml, whereas residual normal CFU-C were found greater than or equal to 1.065 g/ml (P < 0.01). PV clonal and residual normal erythroid burst-forming progenitors were not separable by either method. Thus PV clonal CFU-C are larger and less dense cells than are residual normal CFU-C.
在先前对两名真性红细胞增多症(PV)患者的研究中,这两名患者在X连锁的葡萄糖-6-磷酸脱氢酶(G6PD)位点为杂合子,在非淋巴细胞中仅发现了A型酶。然而,一些体外培养的红系和粒系集落为B型,因此可能源自正常祖细胞。其中一名患者有足够数量的B型集落(8%),从而可以对正常和PV克隆集落形成细胞的物理特性进行研究。当通过单位重力速度沉降分离骨髓细胞时,大多数PV克隆性粒-巨噬细胞祖细胞(CFU-C)(A型G6PD)沉降速度在6.4至7.2毫米/小时之间,而大多数残留的正常B型CFU-C沉降速度小于或等于5.9毫米/小时(P = 0.04)。当血细胞在不连续的浮力密度梯度上分离时,PV克隆性CFU-C在密度<1.065克/毫升处达到平衡,而残留的正常CFU-C则在密度≥1.065克/毫升处被发现(P < 0.01)。PV克隆性和残留的正常红系爆式集落形成祖细胞通过这两种方法均无法分离。因此,PV克隆性CFU-C比残留的正常CFU-C更大且密度更低。