Risk factors for pulmonary hypertension in patients with β thalassemia intermedia

Mehran Karimi, Khaled M. Musallam, Maria Domenica Cappellini, Shahina Daar, Amal El-Beshlawy, Khawla Belhoul, Mohamed Salaheldin Saned, Sally Temraz, Suzanne Koussa, Ali T. Taher

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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 × 10 6/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.

Original languageEnglish
Pages (from-to)607-610
Number of pages4
JournalEuropean Journal of Internal Medicine
Volume22
Issue number6
DOIs
Publication statusPublished - Dec 2011

Keywords

  • Hemolysis
  • Hypercoagulability
  • Pulmonary hypertension
  • Risk factors
  • Thalassemia intermedia
  • Thromboembolism

ASJC Scopus subject areas

  • Internal Medicine

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    Karimi, M., Musallam, K. M., Cappellini, M. D., Daar, S., El-Beshlawy, A., Belhoul, K., Saned, M. S., Temraz, S., Koussa, S., & Taher, A. T. (2011). Risk factors for pulmonary hypertension in patients with β thalassemia intermedia. European Journal of Internal Medicine, 22(6), 607-610. https://doi.org/10.1016/j.ejim.2011.05.013