Polycythaemia Vera: in Vitro Studies of Circulating Erythroid Progenitors
Polycythaemia Vera: in Vitro Studies of Circulating Erythroid Progenitors
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真性红细胞增多症：循环红细胞祖细胞的体外研究
DOI：
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发表时间：
1980
影响因子：
6.5
通讯作者：
B. Varet
中科院分区：
文献类型：
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作者：
Catherine Lacombe;Nicole Casadevall;B. Varet
使用血浆凝块技术培养来自健康受试者、继发性红细胞增多症患者和真性红细胞增多症（PV）患者的外周血单个核细胞，并在14 d后对大红细胞集落或爆发进行评分。在所有20例PV患者中，在未添加促红细胞生成素的情况下出现红细胞爆发，但在正常受试者（n=10）和继发性红细胞增多症患者（n=6）中均未出现红细胞爆发。6例PV患者的促红细胞生成素剂量反应曲线的特征是初始平台期，随后反应接近正常。这表明两个群体的红系祖细胞在PV中共存，一个异常敏感，另一个正常响应促红细胞生成素。在9名患者中，第14天血液中自发爆发与高剂量促红细胞生成素诱导的爆发的比率与第7天相同患者骨髓中自发集落与高剂量促红细胞生成素诱导的集落的比率相同。这反驳了自发性红细胞爆发来自红细胞祖细胞（BFUE）对促红细胞生成素异常敏感的假设。此外，来自健康受试者的血液单核细胞被发现引起红细胞爆发时，促红细胞生成素添加延迟到6天的文化。这与促红细胞生成素在体内通常不需要用于早期（BFUE）至晚期（CFUE）红系祖细胞的分化的概念一致。这些结果表明，在PV中，所有BFUE通常对不同于促红细胞生成素的生理因子有反应，并负责其分化为CFUE，并且对促红细胞生成素的异常反应，红系祖细胞群体的特征，仅在最成熟（CFUE）水平表达。
Peripheral blood mononuclear cells from healthy subjects, patients with secondary polycythaemia and patients with polycythaemia vera (PV) were cultured using the plasma clot technique, and large erythroid colonies or bursts were scored after 14 d. Erythroid bursts appeared without addition of erythropoietin in all 20 patients with PV but never in normal subjects (n=10) nor in patients with secondary polycythaemia (n=6). The erythropoietin dose‐response curves in six PV patients were characterized by an initial plateau followed by a nearly normal response. This suggests the coexistence of two populations of erythroid progenitors in PV, one abnormally sensitive, the other normally responsive to erythropoietin. In nine patients the ratio of spontaneous bursts to bursts induced by high doses of erythropoietin from the blood on day 14 was identical with the ratio of spontaneous colonies to colonies induced by high doses of erythropoietin from the bone marrow of the same patients on day 7. This argues against the hypothesis that spontaneous erythroid bursts are derived from erythroid progenitors (BFUE) abnormally sensitive to erythropoietin. Moreover blood mononuclear cells from healthy subjects were found to give rise to erythroid bursts when erythropoietin addition was delayed until 6 d in culture. This is consistent with the concept that erythropoietin is not normally required in vivo for the differentiation of early (BFUE) to late (CFUE) erythroid progenitors. These findings suggest that in PV all BFUE are normally responsive to the physiological factor(s) different from erythropoietin and responsible for their differentiation into CFUE, and that the abnormal response to erythropoietin, distinctive of a population of erythroid progenitors, is expressed only at the most mature (CFUE) level.