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真性红细胞增多症:体外红细胞生成的激素调节

Polycythemia vera: hormonal modulation of erythropoiesis in vitro.

作者信息

Golde D W, Bersch N, Cline M J

出版信息

Blood. 1977 Mar;49(3):399-405.

PMID:836949
Abstract

We performed in vitro culture studies examining the interaction of erythropoietin with red cell progenitors in polycythemia vera. Bone marrow was obtained from five patients with typical disease and from five healthy volunteers, and assayed for erythroid colony formation (CFU-E) by the methylcellulose technique. In cultures without added erythropoietin, a mean eightfold greater cloning efficiency was noted with the polycythemia vera marrows, as compared to normal. There was prominent stimulation of colony formation by erythropoietin, and the shape of the erythropoietin dose-response cruves appeared to be similar in both patients and controls. Anti-erythropoietin antibody reduced the number of CFU-E in cultures not containing added erythropoietin, but did not eliminate them. Dexamethasone (10(-9) M) caused a consistent increase in CFU-E in the patients' cultures. These studies provide evidence for functional erythropoietin and glucocorticosteroid receptor mechanisms on erythroid precursors in polycythemia vera. The observations are consistent with a concept of this disease as a disorder of hematopoietic stem cells in which peripheral erythrocytosis is caused by an expanded erythroid progenitor compartment which maintains responsiveness to hormonal modulation.

摘要

我们进行了体外培养研究,以检测真性红细胞增多症中促红细胞生成素与红细胞祖细胞的相互作用。从5例典型疾病患者和5名健康志愿者获取骨髓,采用甲基纤维素技术检测红系集落形成(CFU-E)。在未添加促红细胞生成素的培养物中,与正常情况相比,真性红细胞增多症骨髓的克隆效率平均高8倍。促红细胞生成素显著刺激集落形成,患者和对照组中促红细胞生成素剂量反应曲线的形状似乎相似。抗促红细胞生成素抗体减少了未添加促红细胞生成素的培养物中CFU-E的数量,但并未将其消除。地塞米松(10⁻⁹ M)使患者培养物中的CFU-E持续增加。这些研究为真性红细胞增多症中红系前体细胞上功能性促红细胞生成素和糖皮质激素受体机制提供了证据。这些观察结果与将该疾病视为造血干细胞疾病的概念一致,其中外周红细胞增多是由扩大的红系祖细胞区室引起的,该区域保持对激素调节的反应性。

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