Pulmonary hypertension in thalassemia

摘要: Pulmonary hypertension (PH) is common in thalassemia and contributes to mortality. Advancing age a history of splenectomy are major risk factors this population. The etiology PH multifactorial, involving complex interaction platelets, the coagulation system, erythrocytes, endothelial cells along with inflammatory vascular mediators. long-term effect splenectomy, red cell membrane pathology, abnormalities, low nitric oxide (NO) bioavailability, excess arginase activity, platelet activation, oxidative stress, iron overload, chronic hemolysis play role. process disables arginine-NO pathway through simultaneous release erythrocyte cell-free hemoglobin. Both NO its obligate substrate arginine rapidly consumed. biological consequences on bioavailability ultimately translate into clinical manifestations PH. Guidelines for management have not yet been established; however, trials ongoing an effort guide future therapy.