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JAK2V617F allele burden is associated with thrombotic mechanisms activation in polycythemia vera and essential thrombocythemia patients

2014-01Journal article Open access
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dc.contributor.authorCoucelo, M
dc.contributor.authorCaetano, G
dc.contributor.authorSevivas, T
dc.contributor.authorAlmeida Santos, S
dc.contributor.authorFidalgo, T
dc.contributor.authorBento, C
dc.contributor.authorFortuna, M
dc.contributor.authorDuarte, M
dc.contributor.authorMenezes, C
dc.contributor.authorRibeiro, ML
dc.date.accessioned2018-11-28T13:01:24Z
dc.date.available2018-11-28T13:01:24Z
dc.date.issued2014-01
dc.description.abstractThe clinical courses of polycythemia vera (PV) and essential thrombocythemia (ET) are characterized by thrombohemorrhagic diathesis. Several groups have suggested an association between JAK2V617F mutation and thrombosis. We hypothesized a relationship between JAK2V617F allele burden, cellular activation parameters, and thrombosis. We evaluated a group of PV and ET patients using flow cytometry: platelet CD62P, CD63, and dense granules, platelet-leukocyte aggregates (PLA), leukocyte CD11b and monocyte tissue factor (TF) expression. All patients had increased baseline platelet CD62P and CD63 expression (p < 0.05); 71 % of PV and 47 % of ET presented with a storage pool disease. Leukocyte CD11b, TF, and PLA were elevated in all patients. TF was higher in PV compared to ET (p < 0.05) and platelet-neutrophil [polymorphonuclear (PMN)] aggregates were increased in ET versus PV (p < 0.05). In ET, PLA were correlated with platelet numbers (p < 0.05). In all patients, JAK2V617F allele burden was directly correlated with monocyte CD11b. Patients with JAK2V617F allele burden >50 % presented higher levels of leukocyte activation. In ET, thrombosis was associated with JAK2V617F mutation (p < 0.05, χ (2) = 5.2), increased monocyte CD11b (p < 0.05) and with platelet-PMN aggregates (p < 0.05). In ET patients, hydroxyurea does not significantly reduce the activation parameters. Our data demonstrate that JAK2V617F allele burden is directly correlated with activation parameters that drive mechanisms that favor thrombosis.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationInt J Hematol. 2014 Jan;99(1):32-40.pt_PT
dc.identifier.doi10.1007/s12185-013-1475-9pt_PT
dc.identifier.urihttp://hdl.handle.net/10400.4/2183
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.subjectJanus Quinase 2pt_PT
dc.subjectPolicitemia Verapt_PT
dc.subjectTrombocitemia Essencialpt_PT
dc.subjectTrombosept_PT
dc.subjectMutaçãopt_PT
dc.titleJAK2V617F allele burden is associated with thrombotic mechanisms activation in polycythemia vera and essential thrombocythemia patientspt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.endPage40pt_PT
oaire.citation.issue1pt_PT
oaire.citation.startPage32-40pt_PT
oaire.citation.volume99pt_PT
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT

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