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Pulmonary hypertension and β‐thalassemia major: Report of a case, its treatment, and a review of the literature
Author(s) -
Tam Denise H.,
Farber Harrison W.
Publication year - 2006
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.20603
Subject(s) - medicine , pulmonary hypertension , thalassemia , hemosiderosis , beta thalassemia , complication , intensive care medicine , pediatrics
Pulmonary hypertension is a common complication of β‐thalassemia major. We report a case of successful treatment of pulmonary hypertension in a patient with β‐thalassemia major and review the literature on pulmonary hypertension and β‐thalassemia major. A 28‐year‐old man with β‐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 β‐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. Am. J. Hematol. 81:443–447, 2006. © 2006 Wiley‐Liss, Inc.