Cellular distribution of hemoglobin F in a clonal hemopoietic stem-cell disorder.
Cellular distribution of hemoglobin F in a clonal hemopoietic stem-cell disorder.
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克隆性造血干细胞疾病中血红蛋白 F 的细胞分布。
DOI：
10.1056/nejm197801122980203
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发表时间：
1978
期刊：
影响因子：
--
通讯作者：
G. Stamatoyannopoulos
中科院分区：
文献类型：
--
作者：
T. Papayannopoulou;H. Bunn;G. Stamatoyannopoulos
由于慢性粒细胞白血病中的所有红系细胞都被认为是由一种共同的多能干细胞产生的，因此我们研究了该疾病患者胎儿血红蛋白的细胞分布，以测试具有和不具有血红蛋白F的细胞是否具有共同或单独的多能干细胞来源。在一名患有慢性粒细胞白血病和镰状/β地中海贫血的患者中，缓解期17%的红细胞和急变期18%至22%的红细胞含有胎儿血红蛋白。在24名非血红蛋白病性白血病患者中，0.3%至28%的红细胞含有血红蛋白F。由于慢性粒细胞白血病的红细胞几乎完全来自恶性克隆，这些数据表明，含有胎儿血红蛋白的细胞与不含血红蛋白F的细胞来自相同的多能干细胞祖细胞。这些发现反对一种单独的“部分转换”多能干细胞系负责维持成人血红蛋白F的产生。
Because all erythroid cells in chronic myelogenous leukemia are believed to arise from a common pluripotent stem cell, we studied the cellular distribution of fetal hemoglobin in patients with the disease to test whether cells with and without hemoglobin F have a common or separate pluripotent stem-cell origin. In a patient with chronic myelogenous leukemia and sickle/beta thalassemia, 17 per cent of the red cells during remission and 18 to 22 per cent during the blastic phase contained fetal hemoglobin. In 24 non-hemoglobinopathic leukemic patients 0.3 to 28 per cent of red cells contained hemoglobin F. Since the erythrocytes in chronic myelogenous leukemia are almost exclusively derived from the malignant clone, these data suggest that cells containing fetal hemoglobin originate from the same pluripotent stem-cell progenitor as those without hemoglobin F. The findings argue against a separate line of "partially switched" pluripotent stem cells as being responsible for maintenance of hemoglobin F production in the adult.