CHRONIC MYELOCYTIC-LEUKEMIA - CLONAL ORIGIN IN A STEM-CELL COMMON TO GRANULOCYTE, ERYTHROCYTE, PLATELET AND MONOCYTE-MACROPHAGE
CHRONIC MYELOCYTIC-LEUKEMIA - CLONAL ORIGIN IN A STEM-CELL COMMON TO GRANULOCYTE, ERYTHROCYTE, PLATELET AND MONOCYTE-MACROPHAGE
复制标题
DOI：
10.1016/0002-9343(77)90124-3
复制
发表时间：
1977-01-01
影响因子：
5.9
通讯作者：
PAPAYANNOPOULOU, T
中科院分区：
文献类型：
--
作者：
FIALKOW, PJ;JACOBSON, RJ;PAPAYANNOPOULOU, T
本文对8例慢性粒细胞白血病（CML）患者的粒细胞葡萄糖-6-磷酸脱氢酶（G-6-PD）同工酶进行了测定。这些患者在X连锁G-6-PD基因座上的共同基因GdB和变异基因GdA是杂合的，因此在皮肤细胞中发现了B和A酶类型。与正常细胞相比，CML粒细胞中只有1个G-6-PD型。这种单酶表型在CML粒细胞中发现，而在非白血病粒细胞中未发现，这一事实提供了该疾病具有克隆起源的有力证据。在红细胞、血小板和培养的巨噬细胞中也发现了单酶表型，表明这些细胞具有共同的干细胞，这是CML中异常的部位。在1例研究患者中，未发现涉及培养的骨髓成纤维细胞的证据。CML的克隆起源排除了细胞募集作为唯一的发病机制。白血病是由于单个细胞中罕见的初始事件，或克隆中发生的一系列事件导致其演变为CML，或两者兼而有之。
Glucose-6-phosphate dehydrogenase (G-6-PD) isoenzyme types of granulocytes were determined in 8 women with chronic myelocytic leukemia (CML). The patients were heterozygous at the X-linked G-6-PD locus for the common gene, GdB, and a variant, such as GdA, so that B and A enzyme types were found in skin cells. In contrast to these normal cells, only 1 G-6-PD type was found in CML granulocytes. The fact that such single enzyme phenotypes are found in CML granulocytes, but not in nonleukemic granulocytes, provides strong evidence that the disease has a clonal origin. Single enzyme phenotypes were also found in erythrocytes, platelets and cultured macrophages indicating that these cells have a common stem cell which is the site of the abnormality in CML. In the 1 studied patient, no evidence was found for involvement of cultured marrow fibroblasts. Clonal origin of CML excludes cell recruitment as a sole pathogenetic mechanism. The leukemia arises as a consequence of a rare initial event in a single cell, or a series of events occurs in a clone such that it evolves into CML, or both.