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wjmsicuan

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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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