Literature DB >> 16680745

Pulmonary hypertension and beta-thalassemia major: report of a case, its treatment, and a review of the literature.

Denise H Tam1, Harrison W Farber.   

Abstract

Pulmonary hypertension is a common complication of beta-thalassemia major. We report a case of successful treatment of pulmonary hypertension in a patient with beta-thalassemia major and review the literature on pulmonary hypertension and beta-thalassemia major. A 28-year-old man with beta-thalassemia major, splenectomy, hepatitis C, and hemosiderosis who presented with increasing dyspnea on exertion was diagnosed with pulmonary hypertension. After receiving continuous epoprostenol infusion and desferoxamine, his functional capacity and hemodynamic status improved. To our knowledge, this is the first case of pulmonary hypertension associated with beta-thalassemia treated with continuous epoprostenol infusion and desferoxamine. Epoprostenol, beneficial in the treatment of other types of pulmonary hypertension, may ameliorate the morbidity and mortality of pulmonary hypertension associated with thalassemia.

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Year:  2006        PMID: 16680745     DOI: 10.1002/ajh.20603

Source DB:  PubMed          Journal:  Am J Hematol        ISSN: 0361-8609            Impact factor:   10.047


  7 in total

1.  Pulmonary arterial hypertension in a patient with β-thalassemia intermedia and reversal with infusion epoprostenol then transition to oral calcium channel blocker therapy: review of literature.

Authors:  Kamonpun Ussavarungsi; Charles D Burger
Journal:  Pulm Circ       Date:  2014-09       Impact factor: 3.017

2.  Pulmonary hypertension in well-transfused thalassemia major patients.

Authors:  Antonella Meloni; Jon Detterich; Alessia Pepe; Paul Harmatz; Tom D Coates; John C Wood
Journal:  Blood Cells Mol Dis       Date:  2014-11-24       Impact factor: 3.039

3.  Sildenafil therapy in thalassemia patients with Doppler-defined risk of pulmonary hypertension.

Authors:  Claudia R Morris; Hae-Young Kim; John Wood; John B Porter; Elizabeth S Klings; Felicia L Trachtenberg; Nancy Sweeters; Nancy F Olivieri; Janet L Kwiatkowski; Lisa Virzi; Sylvia T Singer; Ali Taher; Ellis J Neufeld; Alexis A Thompson; Vandana Sachdev; Sandra Larkin; Jung H Suh; Frans A Kuypers; Elliott P Vichinsky
Journal:  Haematologica       Date:  2013-04-12       Impact factor: 9.941

Review 4.  Pulmonary hypertension associated with chronic hemolytic anemia and other blood disorders.

Authors:  Roberto F Machado; Harrison W Farber
Journal:  Clin Chest Med       Date:  2013-10-17       Impact factor: 2.878

Review 5.  Pulmonary hypertension associated with thalassemia syndromes.

Authors:  Dustin R Fraidenburg; Roberto F Machado
Journal:  Ann N Y Acad Sci       Date:  2016-03-23       Impact factor: 5.691

Review 6.  How I treat hypoxia in adults with hemoglobinopathies and hemolytic disorders.

Authors:  Evans M Machogu; Roberto F Machado
Journal:  Blood       Date:  2018-09-11       Impact factor: 22.113

7.  Treatment of pulmonary hypertension in three patients with ß-thalassemia intermedia using pulmonary arterial hypertension-specific medications.

Authors:  Demet Menekşe Gerede; Aynur Acıbuca; Tamer Sayın; Çetin Erol
Journal:  Anatol J Cardiol       Date:  2016-01       Impact factor: 1.596

  7 in total

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