Angiogenesis in pulmonary hypertension with myelofibrosis

Zetterberg, Eva; Popat, Uday; Hasselbalch, Hans Carl; Prchal, Josef T.; Palmblad, Jan

doi:10.3324/haematol.12426

Cited by 16 publications

(11 citation statements)

References 6 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 pro-angiogenic phenomenon [14,71]. Other studies demonstrated that distinctive features of myelofibrosis associated with PH include normal or low circulating CD34 cell count, polyclonal platelets and granulocytes, the absence of peripheral blood dacrocytes and the JAK2 1849G>T(V617F) mutation [72,73].…”

Section: Precapillary Pulmonary Hypertensionsupporting

confidence: 53%

Pulmonary hypertension in patients with chronic myeloproliferative disorders

2015

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Pulmonary hypertension (PH) is a major complication of several haematological disorders. Chronic myeloproliferative diseases (CMPDs) associated with pulmonary hypertension have been included in group five of the clinical classification for pulmonary hypertension, corresponding to pulmonary hypertension for which the aetiology is unclear and/or multifactorial. The aim of this review is to discuss the epidemiology, pathogenic mechanism and treatment approaches of the more common forms of pulmonary hypertension in the context of CMPD's: chronic thromboembolic pulmonary hypertension, precapillary pulmonary hypertension and drug-induced PH. @ERSpublications Epidemiology, pathogenic mechanism and treatment approach of PH in patients with chronic myeloproliferative diseases http://ow.ly/Q1QX4

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Section: Precapillary Pulmonary Hypertensionsupporting

confidence: 53%

Pulmonary hypertension in patients with chronic myeloproliferative disorders

2015

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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: Aetiologymentioning

confidence: 66%

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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“…Hematopoietic myeloid proangiogenic progenitors play a central role in endothelial injury and repair. We and others have reported that distinct [2][3][4] or indolent 5 myeloid abnormalities are present in the BM of the majority, if not all, of patients with PAH and even in unaffected family members 5 in familial cases of the disease. These findings and the unexplained high incidence of PAH among patients with myeloproliferative diseases 6,7 suggest a myelopulmonary pathophysiologic link.…”

Section: Introductionmentioning

confidence: 70%

Pulmonary vascular disease in mice xenografted with human BM progenitors from patients with pulmonary arterial hypertension

Asosingh

Farha

Lichtin

et al. 2012

Blood

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Hematopoietic myeloid progenitors released into the circulation are able to promote vascular remodeling through endothelium activation and injury. Endothelial injury is central to the development of pulmonary arterial hypertension (PAH), a proliferative vasculopathy of the pulmonary circulation, but the origin of vascular injury is unknown. In the present study, mice transplanted with BM-derived IntroductionVascular endothelial injury with in situ thrombi is a typical pathologic finding in pulmonary arterial hypertension (PAH). Progressive disease is characterized by complex vascular malformations composed of disorganized proliferating monoclonal endothelial cells with neointima formation. 1 Although endothelial injury is hypothesized to account for the origins of PAH, the underlying mechanism of the vascular injury is unknown. Hematopoietic myeloid proangiogenic progenitors play a central role in endothelial injury and repair. We and others have reported that distinct 2-4 or indolent 5 myeloid abnormalities are present in the BM of the majority, if not all, of patients with PAH and even in unaffected family members 5 in familial cases of the disease. These findings and the unexplained high incidence of PAH among patients with myeloproliferative diseases 6,7 suggest a myelopulmonary pathophysiologic link. In support of this concept, competent hematopoietic progenitors are required for disease development in the monocrotaline-or hypoxia-induced murine models of pulmonary hypertension, and BM transplantation can transfer disease to healthy naive mice. 8 In contrast to developmental origins of blood cells and vascular endothelium from the common hemangioblast, hematopoietic stem cells in the adult do not differentiate into endothelium, but rather promote postnatal angiogenesis and homeostasis in a paracrine fashion. [9][10][11][12] In the hierarchy of adult hematopoietic stem cell differentiation, a small pool of pluripotent, BMresident stem cells exhibit self-renewal and long-term survival. 13 These stem cells proliferate and differentiate into relatively shortlived multipotent progenitors that give rise to common lymphoid and common myeloid progenitors. Common lymphoid progenitors further differentiate into B-or T cell-committed precursors. The common myeloid progenitors proliferate and differentiate into bipotent common erythroid/megakaryocyte progenitors and into multipotent monocyte/granulocyte progenitors. These lineagerestricted myeloid progenitors differentiate into mature blood cells via unilineage-committed intermediate precursors. The hierarchy of proliferation and differentiation is at each bifurcation strictly regulated by lineage-specific transcription factors. 13 In the BM, the cell-surface glycoprotein CD133 is highly expressed on immature progenitors, allowing CD133 expression to define the population of hematopoietic progenitors. Functional analysis of human BM-derived CD133 ϩ cells indicates that this fraction is enriched in primitive multilineage hematopoietic stem cells. 14 Early outgrowth pro...

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Angiogenesis in pulmonary hypertension with myelofibrosis

Cited by 16 publications

References 6 publications

Pulmonary hypertension in patients with chronic myeloproliferative disorders

Pulmonary hypertension in patients with chronic myeloproliferative disorders

Pulmonary hypertension in patients with chronic myeloproliferative disorders

Pulmonary vascular disease in mice xenografted with human BM progenitors from patients with pulmonary arterial hypertension

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