Pulmonary Hypertension Risk In Patients With Hemoglobin H Disease: Low Incidence And Absence Of Correlation With Splenectomy

Pulmonary hypertension (PHT) is a common complication for patients with beta thalassemia intermediate (TI), especially splenectomized patients. However, the frequency and risk factors of PHT in patients with hemoglobin H (HbH) disease is unknown. The purpose of this study was to identify the prevalence of PHT risk manifested as tricuspid regurgitant jet velocity (TRV) >= 2.5 m/s in patients with HbH disease and its correlation with splenectomy. One hundred and ninety-eight patients with HbH disease who visited the 303rd Hospital of the People's Liberation Army (Nanning, China) were investigated. Thirteen subjects (6.5%) were diagnosed as having a risk of PHT. Regression analyses showed that the prevalence of PHT risk was correlated only with age (r = 0.195, p = 0.006) and not with splenectomy. The risk of PHT in patients older than 35 years was 5.7 times (range 1.8-18.6) greater than that for patients younger than 35 years. For splenectomized patients compared to those with HbH disease, patients with TI had a higher frequency of PHT risk, higher nucleated red blood cell counts (46.03 +/- 41.11 x 10(9)/l vs. 0.18 +/- 1.19 x 10(9)/l, p < 0.001) and a higher platelet counts (837.6 +/- 178.9 x 10(9)/l vs. 506.7 +/- 146.2 x p < 0.001). PHT risk is low in patients with HbH disease and does not correlate with splenectomy. Patients older than 35 years should be monitored regularly. Copyright (C) 2013 S. Karger AG, Basel