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肺动脉高压与重型β地中海贫血:一例报告、治疗及文献综述

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

作者信息

Tam Denise H, Farber Harrison W

机构信息

Evans Medical Foundation, Boston Medical Center, Boston University School of Medicine, Boston, Massachusetts 02118, USA.

出版信息

Am J Hematol. 2006 Jun;81(6):443-7. doi: 10.1002/ajh.20603.

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.

摘要

肺动脉高压是重型β地中海贫血的常见并发症。我们报告了1例成功治疗重型β地中海贫血合并肺动脉高压患者的病例,并回顾了有关肺动脉高压和重型β地中海贫血的文献。1例28岁患有重型β地中海贫血、脾切除、丙型肝炎和含铁血黄素沉着症的男性患者,因劳力性呼吸困难加重就诊,被诊断为肺动脉高压。在接受持续静脉输注依前列醇和去铁胺治疗后,其功能状态和血流动力学状况得到改善。据我们所知,这是首例采用持续静脉输注依前列醇和去铁胺治疗的重型β地中海贫血合并肺动脉高压病例。依前列醇对治疗其他类型的肺动脉高压有益,可能会改善地中海贫血相关肺动脉高压的发病率和死亡率。

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