Department of Respirology, Graduate School of Medicine, Chiba University, Japan.
Department of Medicine, School of Medicine, Chiba University, Japan.
Intern Med. 2024 Jun 1;63(11):1585-1590. doi: 10.2169/internalmedicine.2307-23. Epub 2023 Nov 13.
A 51-year-old Thai woman diagnosed with β-thalassemia underwent regular blood transfusion and iron-chelating therapy. However, after voluntarily discontinuing treatment, the patient developed progressive dyspnea and was diagnosed with pulmonary hypertension following right heart catheterization. Despite resuming blood transfusions, her condition did not improve. Because the patient had a history of multiple organ failure, curative treatment for β-thalassemia was not feasible, and macitentan was administered. Despite experiencing hypotension as an adverse event, her condition remained stable during macitentan treatment. Thus, macitentan may be well tolerated in patients with pulmonary hypertension caused by β-thalassemia with multiple organ dysfunction.
一位 51 岁的泰国女性被诊断患有β-地中海贫血症，她接受了定期输血和铁螯合治疗。然而，在自愿停止治疗后，该患者出现进行性呼吸困难，并在进行右心导管检查后被诊断为肺动脉高压。尽管恢复了输血，但她的病情并未改善。由于该患者有多个器官衰竭的病史，因此无法进行β-地中海贫血症的治愈性治疗，给予了马昔腾坦。尽管出现了低血压作为不良反应，但在马昔腾坦治疗期间，她的病情仍然稳定。因此，马昔腾坦可能在伴有多器官功能障碍的β-地中海贫血症引起的肺动脉高压患者中耐受良好。
