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Fifty\u2010three ET patients were studied. Plasma levels of SDF\u20101, IL\u20108 and GRO\u2010\u03b1, evaluated by enzyme\u2010linked immunosorbent assay, and flow cytometric analysis of CXCR1 and CXCR2 on the platelet membrane, were found to be normal in ET patients. CXCR4 expression on platelet surface as well as platelet <jats:italic>CXCR4<\/jats:italic> mRNA detected by real\u2010time reverse transcription polymerase chain reaction, were decreased. Platelet CXCR4 internalization rate was normal while SDF\u20101\u2010induced platelet aggregation was delayed, decreased or absent. Immunohistochemical staining revealed that megakaryocytes were also affected. CXCR4 decrease was not observed either in peripheral white blood cells or in circulating CD34<jats:sup>+<\/jats:sup> precursors. These results show that CXCR4 is decreased in the megakaryocytic lineage in ET, mainly due to a reduced CXCR4 production, and an abnormal platelet response to SDF\u20101. This report is the first to describe platelet and megakaryocytic CXCR4 deficiency in a human disease and the presence of this abnormality in a megakaryocytic\u2010related illness highlights the important role of SDF\u20101\/CXCR4 axis in platelet development.<\/jats:p>","DOI":"10.1111\/j.1365-2141.2008.07428.x","type":"journal-article","created":{"date-parts":[[2008,11,6]],"date-time":"2008-11-06T02:23:22Z","timestamp":1225938202000},"page":"69-77","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Dysregulation of stromal derived factor 1\/CXCR4 axis in the megakaryocytic lineage in essential thrombocythemia"],"prefix":"10.1111","volume":"144","author":[{"given":"Juan P.","family":"Salim","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nora 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