Hematological Disorders and Pulmonary Hypertension

Authors

Rajamma Mathew, New York Medical CollegeFollow
Jing Huang
Joseph M. Wu, New York Medical CollegeFollow
John T. Fallon, New York Medical CollegeFollow
Michael H. Gewitz, New York Medical CollegeFollow

First Page

703

Last Page

718

Document Type

Article

Publication Date

12-26-2016

Department

Pediatrics

Second Department

Biochemistry and Molecular Biology

Third Department

Pathology, Microbiology and Immunology

Abstract

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.

Recommended Citation

Mathew, R., Huang, J., Wu, J. M., Fallon, J. T., & Gewitz, M. H. (2016). Hematological disorders and pulmonary hypertension. World Journal of Cardiology, 8(12), 703-718. doi:10.4330/wjc.v8.i12.703

Publisher's Statement

Originally published in World Journal of Cardiology. Licensed under CC-BY-NC 4.0. https://doi.org/10.4330/wjc.v8.i12.703