Pathogenesis of pulmonary hypertension in patients with hemolytic disorders.

The vessel shown in the figure is an autopsy specimen from a 55-year-old male with sickle cell disease and pulmonary hypertension and demonstrates the intimal and medial pulmonary arterial proliferative vasculopathy characteristic of the disease. Mechanistic factors related to hemolytic anemia and systemic complications of sickle cell disease that may contribute to the development of this vasculopathy are shown around the vessel.

Abbreviations: NO, nitric oxide; ET-1 endothelin 1; HIF, hypoxia inducible factor; EPO, erythropoietin; VEGF, vascular endothelial growth factor; PS, phosphatidylserine; TF, tissue factor; Q, lung perfusion.

Figure reproduced from Machado et al. with permission⁵⁹