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In vitro megakaryocyte differentiation and proplatelet formation in ph-negative classical myeloproliferative neoplasms: distinct patterns in the different clinical phenotypes

Balduini, Alessandra and Badalucco, Stefania and Pugliano, Maria Teresa and Baev, Denis and De Silvestri, Annalisa and Cattaneo, Marco and Rosti, Vittorio and Barosi, Giovanni (2011) In vitro megakaryocyte differentiation and proplatelet formation in ph-negative classical myeloproliferative neoplasms: distinct patterns in the different clinical phenotypes. PLoS One, Vol. 6 (6), e21015. ISSN 1932-6203. Article.

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DOI: 10.1371/journal.pone.0021015

Abstract

Background: Ph-negative myeloproliferative neoplasms (MPNs) are clonal disorders that include primary myelofibrosis (PMF), polycythemia vera (PV) and essential thrombocythemia (ET). Although the pathogenesis of MPNs is still incompletely understood, an involvement of the megakaryocyte lineage is a distinctive feature.
Methodology/Principal Findings: We analyzed the in vitro megakaryocyte differentiation and proplatelet formation in 30 PMF, 8 ET, 8 PV patients, and 17 healthy controls (CTRL). Megakaryocytes were differentiated from peripheral blood CD34+ or CD45+ cells in the presence of thrombopoietin. Megakaryocyte output was higher in MPN patients than in CTRL with no correlation with the JAK2 V617F mutation. PMF-derived megakaryocytes displayed nuclei with a bulbous appearance, were smaller than ET- or PV-derived megakaryocytes and formed proplatelets that presented several structural alterations. In contrast, ET- and PV-derived megakaryocytes produced more proplatelets with a striking increase in bifurcations and tips compared to both control and PMF. Proplatelets formation was correlated with platelet counts in patient peripheral blood. Patients with pre-fibrotic PMF had a pattern of megakaryocyte proliferation and proplatelet formation that was similar to that of fibrotic PMF and different from that of ET.
Conclusions/Significance: In conclusion, MPNs are associated with high megakaryocyte proliferative potential. Profound differences in megakaryocyte morphology and proplatelet formation distinguish PMF, both fibrotic and prefibrotic, from ET and PV.

Item Type:Article
ID Code:6237
Status:Published
Refereed:Yes
Uncontrolled Keywords:Primary myelofibrosis (PMF), essential thrombocythemia (ET), platelet release; disorders
Subjects:Area 05 - Scienze biologiche > BIO/11 Biologia molecolare
Divisions:002 Altri enti e centri di ricerca del Nord Sardegna > ViroStatics, Sassari
Publisher:Public Library of Science
ISSN:1932-6203
Copyright Holders:© 2011 Balduini et al.
Deposited On:11 Jul 2011 09:53

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