Pulmonary arterial hypertension in primary myelofibrosis is common and associated with an altered angiogenic status

Cortelezzi, Agostino; Gritti, Giuseppe; Papa, Nicoletta Del; Pasquini, Maria Cristina; Calori, Rossella; Gianelli, Umberto; Cortiana, M.; Parati, Gianfranco; Onida, Francesco; Sozzi, Fabiola B.; Vener, Claudia; Bianchi, Paola; Deliliers, G Lambertenghi

doi:10.1038/sj.leu.2404943

Cited by 50 publications

(46 citation statements)

References 11 publications

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“…In this study patients with primary myelofibrosis and PH had higher bone marrow microvessel density and vascular endothelial growth factor levels suggesting the presence of a proangiogenic phenomenon [98,99]. Other studies demonstrated that distinctive features of myelofibrosis associated with PH include normal or low circulating CD34 cell count, polyclonal platelets and granulocytes, absence of peripheral blood dacrocytes and the JAK2 1849G.T(V617F) mutation [100,101].…”

Section: Aetiologysupporting

confidence: 54%

“…The study group consisted of 36 patients, 22 with primary myelofibrosis, seven with myelofibrosis developing from polycythaemia vera and seven with myelofibrosis progressing from essential thrombocytosis. PH (Prvs .35 mmHg on transthoracic echocardiography) was found in 13 (36%) patients [13].…”

Section: Epidemiologymentioning

confidence: 97%

“…The possible association of PH with CMPD has been suggested by case reports and small case series [9,10,[11][12][13][14][15][16][17][18][19][20][21][22]. However, the exact incidence and prevalence of PH in this population remains poorly defined.…”

Section: Epidemiologymentioning

confidence: 99%

“…There was no relationship between PH and age at diagnosis, duration of disease, platelet count and haematocrit level. Since angiogenesis is believed to contribute to the pathogenesis of both PH and CMPD, a recent study has investigated the association of PH and primary myelofibrosis [13]. The study group consisted of 36 patients, 22 with primary myelofibrosis, seven with myelofibrosis developing from polycythaemia vera and seven with myelofibrosis progressing from essential thrombocytosis.…”

Section: Epidemiologymentioning

confidence: 99%

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Pulmonary hypertension in patients with chronic myeloproliferative disorders

Adir

Humbert²

2010

Eur Respir J

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Pulmonary hypertension (PH) is a severe haemodynamic disorder in which the pulmonary artery pressure is persistently elevated, leading to right-sided heart failure and death. Recently, chronic myeloproliferative diseases associated with pulmonary hypertension were included in the group 5 category, corresponding to PH for which the aetiology is unclear and/or multifactorial.In this review we will describe the distinct forms of PH in the context of the myeloproliferative diseases chronic thromboembolic pulmonary hypertension and pre-capillary PH mimicking pulmonary arterial hypertension. The epidemiology, mechanisms and treatment approaches will be discussed.

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Section: Aetiologysupporting

confidence: 54%

Section: Epidemiologymentioning

confidence: 97%

Section: Epidemiologymentioning

confidence: 99%

Section: Epidemiologymentioning

confidence: 99%

See 2 more Smart Citations

Pulmonary hypertension in patients with chronic myeloproliferative disorders

Adir

Humbert²

2010

Eur Respir J

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“…However, there are differences as well: patients with MF and PAH have a higher level of serum VEGF and lower levels of circulating endothelial progenitor cells (EPC) than myeloproliferative MF patients. 2 Distinctive features of MF associated with PAH include normal or low circulating CD34 cell count, polyclonal platelets and granulocytes, absence of peripheral blood dacrocytes and the JAK2 1849G>T(V617F) mutation. 3,4 We have recently shown that there is an overexpression of pericytes around the wide and tortuous capillaries in the bone marrow of patients with PMF, PPMF and PTMF, 5 i.e.…”

Section: Angiogenesis In Pulmonary Hypertension With Myelofibrosismentioning

confidence: 99%

Angiogenesis in pulmonary hypertension with myelofibrosis

Zetterberg¹,

Popat²,

Hasselbalch³

et al. 2008

Haematologica

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Circulating and progenitor endothelial cells are abnormal in patients with different types of von Willebrand disease and correlate with markers of angiogenesis

et al. 2011

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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-derived 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 (n 5 22), VWD2A (n 5 9), VWD2B (n 5 19), VWD2M (n 5 17), and VWD3 (n 5 7). Healthy individuals (n 5 20) were used as controls. CEC (CD146 1 , CD31 1 , and CD45 2 ) and EPC (CD34 1 , CD133 1 , and CD45 2 ) were evaluated by flow cytometry. Circulating serum levels of VEGF, E-selectin, P-selectin, EPO, and TPO were determined by ELISA. CEC, VEGF, E-selectin, and EPO were higher and EPC lower in VWD patients than in controls (P < 0.01). Among the five groups of VWD patients and controls, a significant difference was found for CEC (one-way ANOVA: P 5 0.005), EPC (P 5 0.001), E-Selectin (P < 0.0001), EPO (P 5 0.021), and TPO (P 5 0.004): the latter was high in VWD3 patients. In VWD1, we found an inverse relationship between CEC and VWF:Ag levels (P 5 0.048; R 2 5 0.19). Based on these data, CEC are increased in VWD and are associated with the high levels of cytokines involved in angiogenesis (up-regulation). EPC are decreased, suggesting down-regulation of bone marrow-derived angiogenesis in VWD. Am. J. Hematol. 86:650-656, 2011. V

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Pulmonary arterial hypertension in primary myelofibrosis is common and associated with an altered angiogenic status

Cited by 50 publications

References 11 publications

Pulmonary hypertension in patients with chronic myeloproliferative disorders

Pulmonary hypertension in patients with chronic myeloproliferative disorders

Angiogenesis in pulmonary hypertension with myelofibrosis

Circulating and progenitor endothelial cells are abnormal in patients with different types of von Willebrand disease and correlate with markers of angiogenesis

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