Risk factors for pulmonary hypertension in patients with β thalassemia intermedia

      Abstract

      Background

      Pulmonary hypertension (PHT) is a common yet poorly understood complication of β thalassemia intermedia (TI).

      Methods

      We herein evaluated risk factors for PHT in TI, through comparing 64 TI patients with evidence of PHT by symptomatology and echocardiography (Group I) to age- and sex-matched TI patients without PHT (Group II). Retrieved data included demographics, laboratory parameters, clinical characteristics, and received treatments that may influence PHT development; and reflected the period prior to PHT occurrence in Group I.

      Results

      The mean age of Group I patients at development of PHT was 37.3±10.6 years; with 44% being males. Among studied parameters, Group I patients were more likely to be splenectomized (4.9-times), transfusion-naive (3.5-times); hydroxyurea-naive (2.6-times), or iron chelation-naive (2.3-times); and have nucleated red blood cell count ≥300×106/l (2.59-times) or a previous history of thromboembolic events (3.69-times).

      Conclusion

      TI patients who eventually develop PHT may be identified early on by being splenectomized, having high nucleated red blood cell counts and a previous history of thromboembolism. Prospective clinical trials that evaluate the efficacy, safety, and cost effectiveness of transfusion, iron chelation, and hydroxyurea therapy in preventing PHT in TI are invited.

      Keywords

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