Pulmonary hypertension in patients with chronic myeloproliferative disorders

Adir, Yochai; Humbert, Marc

doi:10.1183/09031936.00175909

Cited by 46 publications

(38 citation statements)

References 113 publications

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“…This case is consistent with what is known regarding chronic myeloproliferative disorders associated with pulmonary hypertension, whereby there are multiple attributable causes described [41], similar to the noted heterogeneous etiologies of pulmonary hypertension after treatment with bone marrow transplant [42,43]. …”

Section: Pulmonary Hypertension and Myeloid Cell Disorderssupporting

confidence: 89%

Myeloid-Derived Suppressor Cells and Pulmonary Hypertension

Bryant

Mehrad

Brusko

et al. 2018

IJMS

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Myeloid–derived suppressor cells (MDSCs) comprised a heterogeneous subset of bone marrow–derived myeloid cells, best studied in cancer research, that are increasingly implicated in the pathogenesis of pulmonary vascular remodeling and the development of pulmonary hypertension. Stem cell transplantation represents one extreme interventional strategy for ablating the myeloid compartment but poses a number of translational challenges. There remains an outstanding need for additional therapeutic targets to impact MDSC function, including the potential to alter interactions with innate and adaptive immune subsets, or alternatively, alter trafficking receptors, metabolic pathways, and transcription factor signaling with readily available and safe drugs. In this review, we summarize the current literature on the role of myeloid cells in the development of pulmonary hypertension, first in pulmonary circulation changes associated with myelodysplastic syndromes, and then by examining intrinsic myeloid cell changes that contribute to disease progression in pulmonary hypertension. We then outline several tractable targets and pathways relevant to pulmonary hypertension via MDSC regulation. Identifying these MDSC-regulated effectors is part of an ongoing effort to impact the field of pulmonary hypertension research through identification of myeloid compartment-specific therapeutic applications in the treatment of pulmonary vasculopathies.

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Section: Pulmonary Hypertension and Myeloid Cell Disorderssupporting

confidence: 89%

Myeloid-Derived Suppressor Cells and Pulmonary Hypertension

Bryant

Mehrad

Brusko

et al. 2018

IJMS

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“…CMPD originate in multipotent hematopoietic progenitor cells that are characterized by increases in one or more types of blood cells. CMPD include polycythemia vera, essential thrombocythemia, idiopathic myelofibrosis and chronic myeloid leukemia (CML)[86]. Dingli et al[87] examined 26 patients with CMPD and echocardiography based diagnosis of PH (estimated systolic pulmonary artery pressure 35-100 mmHg); 24 patients had symptoms related to PH and 4 had had splenectomy.…”

Section: Hematological Disorders and Phmentioning

confidence: 99%

Hematological disorders and pulmonary hypertension

Mathew

Huang

et al. 2016

WJC

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Pulmonary hypertension (PH), a serious disorder with a high morbidity and mortality rate, is known to occur in a number of unrelated systemic diseases. Several hematological disorders such as sickle cell disease, thalassemia and myeloproliferative diseases develop PH which worsens the prognosis. Associated oxidant injury and vascular inflammation cause endothelial damage and dysfunction. Pulmonary vascular endothelial damage/dysfunction is an early event in PH resulting in the loss of vascular reactivity, activation of proliferative and antiapoptotic pathways leading to vascular remodeling, elevated pulmonary artery pressure, right ventricular hypertrophy and premature death. Hemolysis observed in hematological disorders leads to free hemoglobin which rapidly scavenges nitric oxide (NO), limiting its bioavailability, and leading to endothelial dysfunction. In addition, hemolysis releases arginase into the circulation which converts L-arginine to ornithine, thus bypassing NO production. Furthermore, treatments for hematological disorders such as immunosuppressive therapy, splenectomy, bone marrow transplantation, and radiation have been shown to contribute to the development of PH. Recent studies have shown deregulated iron homeostasis in patients with cardiopulmonary diseases including pulmonary arterial hypertension (PAH). Several studies have reported low iron levels in patients with idiopathic PAH, and iron deficiency is an important risk factor. This article reviews PH associated with hematological disorders and its mechanism; and iron homeostasis and its relevance to PH.

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“…Cortelezzi et al 3 report the obstruction of pulmonary vessels by circulating megakaryocytes, smooth muscle hyperplasia due to platelet-derived growth factor (PDGF), and altered angiogenic status as potential causes. Adir et al and Guilpain et al 10,11 primarily refer to an increased risk for thromboembolic events, and thus a high risk of pulmonary embolism leading to PH. Extramedullary haematopoiesis, which is a well-known complication of PMF, may also cause PH.…”

Section: Introductionmentioning

confidence: 99%

Frequency and etiology of pulmonary hypertension in patients with myeloproliferative neoplasms

Brabrand

Hansen

Laursen

et al. 2019

European J of Haematology

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Objective Pulmonary hypertension (PH) has been reported to be associated with myeloproliferative neoplasms (MPN), affecting 5%‐48% of MPN patients. With the aims to describe the prevalence of PH in Ph‐MPN patients and explore the cause in identified subjects, we performed a prospective cohort study of Ph‐MPN patients. Method Transthoracic echocardiography (TTE) was performed on all patients. When the TTE was abnormal, further investigations were performed according to current guidelines from the European Society of Cardiology. The primary endpoint was the frequency of PH. The secondary endpoint was causes of PH. Results We included 158 patients, median age was 65 years. Fifty percent had polycythemia vera, 34% essential thrombocytosis, and 11% primary myelofibrosis, 3% post‐ET‐myelofibrosis, and 2% post‐PV‐myelofibrosis. Only six patients (3.8%) were found to have a high probability of PH. They were all examined with right heart catheterization and all met the invasive criteria for PH. In all six patients other causes than MPN for PH were identified, although some contribution from the MPN could not be ruled out in three patients. Conclusion In the largest study ever reported, we found a lower prevalence of PH (3.8%) than previously reported. Screening for PH in unselected MPN patients is not justified.

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Pulmonary hypertension in patients with chronic myeloproliferative disorders

Cited by 46 publications

References 113 publications

Myeloid-Derived Suppressor Cells and Pulmonary Hypertension

Myeloid-Derived Suppressor Cells and Pulmonary Hypertension

Hematological disorders and pulmonary hypertension

Frequency and etiology of pulmonary hypertension in patients with myeloproliferative neoplasms

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