Mathew R, Huang J, Wu JM, Fallon JT, Gewitz MH.
Hematological disorders and pulmonary hypertension.

World J Cardiol. 2016 Dec 26;8(12):703-718.

In this nice review, Mathew et al summarizes the pathophysiologicla mechanisms of pulmonary hypertention  underlying in several hematological disorders, such as sickle cell disease, thalassemia and myeloproliferative diseases. The accompanying pulmonary hypertention to hematological diseases worsens the prognosis. 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. Besides the hemolysis scavenges nitric oxide (NO), limiting its bioavailability, and leading to endothelial dysfunction. Additionaly, the treatments for hematological disorders such as immunosuppressive therapy, splenectomy, bone marrow transplantation, and radiation have been shown to contribute to the development of PH.