Pulmonary Artery Pressure Correlates Directly with Spleen Volume in Non-Splenectomized Hemoglobin E/β-Thalassemia Patients

Prevalence and risk factors for pulmonary hypertension in patients with hemoglobin E/β‐thalassemia disease

“…Values of PASP in patients with an intact spleen were lower than those without one (40.3 ± 4.7 and 48.0 ± 11 mmHg ( P = 0.004), respectively), which is in keeping with a previous report showing mild PHT in non‐splenectomized patients to be due to an increased blood volume as a physiologic adaptation to chronic anemia, a vastly expanded bone marrow space and splenomegaly . However, an increased pulmonary vascular resistance, which varied mostly according to the extent of thrombotic pulmonary arteriopathy, was the major contributing factor to the relatively more severe disease in the splenectomized counterparts .…”

“…The role of sVCAM‐1 as a risk factor for PHT was seen only in splenectomized patients, because its serum levels were different in these patients with and without PHT (1920.3 ± 600.6 ng/mL and 1325.3 ± 365.4 ng/mL, respectively, P = 0.001), but not in their non‐splenectomized counterparts (1847.6 ± 478.9 ng/mL and 1549.6 ± 731.2 ng/mL, respectively, P = 0.280), suggesting different pathogenesis of disease in the two groups, which is in agreement with previous reports .…”