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increase in the urinary Ep levels(102,103).A selective increase in the red cell concentration is a primary clinical characteristic of polycythemia secondary to increased Ep production,since the white cell and platelet counts are unaffected by the hormone.Two families with an inherited recessive polycythemia have been found to have an inappropriate increase in Ep production,which is due to a cellular defect at the site of Ep synthesis(104).No increase in urinary Ep was seen in either family in response to phlebotomy.
In polycythemia vera an inappropriate increase in erythropoiesis occurs without increased Ep production.Urinary Ep ease in hemoglobin or DNA synthesis was quite small when compared with normal marrow cells,but returned to normal once the patients were put into remission with busulfan therapy(105,106).These experiments were consistent with the hypothesis that two cell lines were present;an abnormal clone that was relatively insensitive to Ep and that proliferate autonomously,and a normal cell line that was regulated by Ep and expressed itself only after treatment of the disease with busulfan.This concept of the clonal nature of polycythemia vera has been further supported by

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1楼 2011-03-07 08:19:07

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increase in the urinary EP levels(102,103). 增加尿红细胞生成素水平（102，103）。A selective increase in the red cell concentration is a primary clinical characteristic of polycythemia secondary to increased EP production, since the white cell and platelet counts are unaffected by the hormone. 由于白细胞和血小板计数不受急速的影响.红细胞浓度的选择性增加，是红细胞增多症继发于红细胞生成素生成增加的首要临床特点。Two families with an inherited recessive polycythemia have been found to have an inappropriate increase in EP production,which is due to a cellular defect at the site of EP synthesis(104). 两个患有隐性遗传性红细胞增多症家庭中已发现有不适宜的红细胞生成素生成的增加，这是由于在红细胞生成素位点的细胞缺陷所致（104）。No increase in urinary EP was seen in either family in response to phlebotomy，在进行放血刺激时，两个家庭都没观察到尿红细胞生成素的增加。

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2楼2011-03-07 09:28:35

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ringzhu(金币+20, 翻译EPI+1): ~~ 2011-03-22 17:32:51

In polycythemia vera an inappropriate increase in erythropoiesis occurs without increased EP production. 在真性红细胞增多症，确实有在红细胞生成素生成不增加的不适宜的红细胞生成增加。Urinary EP ease in hemoglobin or DNA synthesis was quite small when compared with normal marrow cells, but returned to normal once the patients were put into remission with busulfan therapy(105,106). 与正常骨髓细胞相比，尿红细胞生成素在血红蛋白或DNA合成时的下降是相当小的，但一旦病人接受马利兰治疗病情缓解时，就恢复了正常（105，106）。 These experiments were consistent with the hypothesis that two cell lines were present;an abnormal clone that was relatively insensitive to EP and that proliferate autonomously, and a normal cell line that was regulated by EP and expressed itself only after treatment of the disease with busulfan. 这些实验与存在两个细胞系的假设一致：一种不正常的克隆对红细胞生成素相对不敏感，能自行增殖；正常的细胞系受红细胞生成素的调控，只有在患者接受马利兰治疗之后才自行表达。 This concEPt of the clonal nature of polycythemia vera has been further supported by这种对真性红细胞增多症克隆性质的概念得到了进一步的支持

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3楼2011-03-07 09:50:44

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