{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,14]],"date-time":"2026-07-14T06:35:13Z","timestamp":1784010913214,"version":"3.55.0"},"reference-count":34,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2011,5,31]],"date-time":"2011-05-31T00:00:00Z","timestamp":1306800000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2011,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>von Willebrand disease (VWD) is the most common inherited bleeding disorder and is caused by quantitative or qualitative defects of von Willebrand factor (VWF). VWF, synthesized by endothelium and megakaryocytes (MK), circulates in plasma and is present in subendothelium and platelets. Circulating endothelial cells (CEC) and progenitor endothelial cells (EPC) have been recently proposed as markers of peripheral and bone marrow\u2010derived angiogenesis. To evaluate the association of CEC\/EPC with known inherited defects of cellular and circulating VWF, we have measured the number of CEC\/EPC together with cytokines involved in angiogenesis in different VWD types. A group of 74 patients was composed by the following VWD types: VWD1 (<jats:italic>n<\/jats:italic> = 22), VWD2A (<jats:italic>n<\/jats:italic> = 9), VWD2B (<jats:italic>n<\/jats:italic> = 19), VWD2M (<jats:italic>n<\/jats:italic> = 17), and VWD3 (<jats:italic>n<\/jats:italic> = 7). Healthy individuals (<jats:italic>n<\/jats:italic> = 20) were used as controls. CEC (CD146<jats:sup>+<\/jats:sup>, CD31<jats:sup>+<\/jats:sup>, and CD45<jats:sup>\u2212<\/jats:sup>) and EPC (CD34<jats:sup>+<\/jats:sup>, CD133<jats:sup>+<\/jats:sup>, and CD45<jats:sup>\u2212<\/jats:sup>) were evaluated by flow cytometry. Circulating serum levels of VEGF, E\u2010selectin, P\u2010selectin, EPO, and TPO were determined by ELISA. CEC, VEGF, E\u2010selectin, and EPO were higher and EPC lower in VWD patients than in controls (<jats:italic>P<\/jats:italic> &lt; 0.01). Among the five groups of VWD patients and controls, a significant difference was found for CEC (one\u2010way ANOVA: <jats:italic>P<\/jats:italic> = 0.005), EPC (<jats:italic>P<\/jats:italic> = 0.001), E\u2010Selectin (<jats:italic>P<\/jats:italic> &lt; 0.0001), EPO (<jats:italic>P<\/jats:italic> = 0.021), and TPO (<jats:italic>P<\/jats:italic> = 0.004): the latter was high in VWD3 patients. In VWD1, we found an inverse relationship between CEC and VWF:Ag levels (<jats:italic>P<\/jats:italic> = 0.048; <jats:italic>R<\/jats:italic><jats:sup>2<\/jats:sup> = 0.19). Based on these data, CEC are increased in VWD and are associated with the high levels of cytokines involved in angiogenesis (up\u2010regulation). EPC are decreased, suggesting down\u2010regulation of bone marrow\u2010derived angiogenesis in VWD. Am. J. Hematol. 2011. \u00a9 2011 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajh.22070","type":"journal-article","created":{"date-parts":[[2011,4,29]],"date-time":"2011-04-29T18:38:35Z","timestamp":1304102315000},"page":"650-656","source":"Crossref","is-referenced-by-count":17,"title":["Circulating and progenitor endothelial cells are abnormal in patients with different types of von Willebrand disease and correlate with markers of angiogenesis"],"prefix":"10.1002","volume":"86","author":[{"given":"Giuseppe","family":"Gritti","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Agostino","family":"Cortelezzi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Bucciarelli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Rezzonico","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Lonati","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"La Marca","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilaria","family":"Silvestris","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Augusto B.","family":"Federici","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2011,5,31]]},"reference":[{"key":"e_1_2_6_2_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2006.02146.x"},{"key":"e_1_2_6_3_2","doi-asserted-by":"publisher","DOI":"10.1160\/TH07-04-0279"},{"key":"e_1_2_6_4_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2008-02-078170"},{"key":"e_1_2_6_5_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2004-02-0769"},{"key":"e_1_2_6_6_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-03-209205"},{"key":"e_1_2_6_7_2","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2009.012658"},{"key":"e_1_2_6_8_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-07-231886"},{"key":"e_1_2_6_9_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2009.03371.x"},{"key":"e_1_2_6_10_2","doi-asserted-by":"publisher","DOI":"10.1160\/TH04-09-0578"},{"key":"e_1_2_6_11_2","doi-asserted-by":"publisher","DOI":"10.1038\/nrc1971"},{"key":"e_1_2_6_12_2","doi-asserted-by":"publisher","DOI":"10.1002\/art.20116"},{"key":"e_1_2_6_13_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V93.9.2951"},{"key":"e_1_2_6_14_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199711273372203"},{"key":"e_1_2_6_15_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.RES.0000137877.89448.78"},{"key":"e_1_2_6_16_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa043814"},{"key":"e_1_2_6_17_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa022287"},{"key":"e_1_2_6_18_2","doi-asserted-by":"publisher","DOI":"10.1161\/hh1301.093953"},{"key":"e_1_2_6_19_2","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2005.09.021"},{"key":"e_1_2_6_20_2","doi-asserted-by":"publisher","DOI":"10.1080\/10428190500144235"},{"key":"e_1_2_6_21_2","first-page":"e47","article-title":"Hematopoietic and endothelial progenitor cell trafficking in patients with myeloproliferative diseases","volume":"92","author":"Oppliger Leibundgut E","year":"2007","journal-title":"Haematologica"},{"key":"e_1_2_6_22_2","doi-asserted-by":"publisher","DOI":"10.1038\/sj.leu.2404943"},{"key":"e_1_2_6_23_2","doi-asserted-by":"publisher","DOI":"10.1038\/sj.leu.2405069"},{"key":"e_1_2_6_24_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-11-123091"},{"key":"e_1_2_6_25_2","doi-asserted-by":"publisher","DOI":"10.1002\/hon.865"},{"key":"e_1_2_6_26_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2008.07519.x"},{"key":"e_1_2_6_27_2","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-08-0432"},{"key":"e_1_2_6_28_2","first-page":"1323","article-title":"Simvastatin reduces endothelial activation and damage but is partially ineffective in inducing endothelial repair in systemic sclerosis","volume":"35","author":"Del Papa N","year":"2008","journal-title":"J Rheumatol"},{"key":"e_1_2_6_29_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.0000077912.12202.FC"},{"key":"e_1_2_6_30_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.0000120705.55512.EC"},{"key":"e_1_2_6_31_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.RES.51.5.587"},{"key":"e_1_2_6_32_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.ATV.5.4.366"},{"key":"e_1_2_6_33_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.ATV.18.2.323"},{"key":"e_1_2_6_34_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.0000112567.53841.10"},{"key":"e_1_2_6_35_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.061307098"}],"container-title":["American Journal of Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajh.22070","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajh.22070","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,21]],"date-time":"2023-11-21T16:23:43Z","timestamp":1700583823000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajh.22070"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,5,31]]},"references-count":34,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2011,8]]}},"alternative-id":["10.1002\/ajh.22070"],"URL":"https:\/\/doi.org\/10.1002\/ajh.22070","archive":["Portico"],"relation":{},"ISSN":["0361-8609","1096-8652"],"issn-type":[{"value":"0361-8609","type":"print"},{"value":"1096-8652","type":"electronic"}],"subject":[],"published":{"date-parts":[[2011,5,31]]}}}