Polycythemia vera. The in vitro response of normal and abnormal stem cell lines to erythropoietin.
Polycythemia vera. The in vitro response of normal and abnormal stem cell lines to erythropoietin.
复制标题
真性红细胞增多症。
DOI：
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发表时间：
1978
影响因子：
15.9
通讯作者：
P. Fialkow
中科院分区：
文献类型：
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作者：
J. Prchal;J. Adamson;S. Murphy;L. Steinmann;P. Fialkow
培养真性红细胞增多症的两个葡萄糖-6-磷酸脱氢酶（G-6-PD）杂合子的骨髓细胞，以确定是否存在非真性红细胞增多症克隆的祖细胞，如果存在，哪些细胞系在添加促红细胞生成素（ESF）后观察到红细胞菌落的增加。为此，测定了单个红系菌落的G-6-PD同工酶活性。在不添加ESF的培养基中分析的所有红系菌落都含有异常克隆的G-6-PD同工酶型特征。然而，随着培养物中ESF浓度的升高，非真性红细胞增多症克隆的菌落增加。对不同类型菌落比例的分析表明，正常细胞和真性红细胞增多症细胞能够在体外对ESF产生反应。在选定的患者中，该技术允许分析正常细胞与异常细胞在病程、治疗反应和晚期并发症（如骨髓纤维化或白血病转化）期间的比例。
Bone marrow cells from two glucose-6-phosphate dehydrogenase (G-6-PD) heterozygotes with polycythemia vera were cultured to determine whether progenitors which wre not of the polycythemia vera clone were present, and, if present, which cell lines contributed to the increase in erythroid colonies observed in response to added erythropoietin (ESF). To accomplish this, the G-6-PD isoenzyme activity of individual erythroid colonies was determined. All of the erythroid colonies analyzed in cultures without added ESF, contained the G-6-PD isoenzyme type characteristic of the abnormal clone. With higher ESF concentrations in the culture, however, there was an increase in the colonies that were not of the polycythemia vera clone. Analysis of the ratio of the various types of colonies indicated that normal and polycythemia vera cells are capable of responding to ESF in vitro. In selected patients, this technique permits analysis of the ratios of normal to abnormal cells during the course of the disease, in response to therapy and during late complications, such as myelofibrosis or leukemic transformation.