Kobayashi Takayuki, Shigeta Ayako, Terada Jiro, Tanabe Nobuhiro, Sugiura Toshihiko, Sakao Seiichiro, Taniguchi Kohei, Oto Takahiro, Tatsumi Koichiro
Department of Respirology, Graduate School of Medicine, Chiba University, Chiba, Japan.
Department of Pulmonary Hypertention Center, Chibaken Saiseikai Narahshino Hospital, Chiba, Japan.
Pulm Circ. 2020 Nov 20;10(4):2045894020969103. doi: 10.1177/2045894020969103. eCollection 2020 Oct-Dec.
While the prognosis of idiopathic pulmonary arterial hypertension has improved significantly due to newer medications, lung transplantation remains a critical therapeutic option for severe pulmonary arterial hypertension. Hence, it is essential for patients awaiting lung transplantation to avoid complications, including thrombocytopenia, which may affect their surgical outcomes. Herein we present the case of a 21-year-old woman diagnosed with idiopathic pulmonary arterial hypertension at the age of 15. She developed thrombocytopenia while awaiting lung transplantation. Her medication was switched from epoprostenol to treprostinil, suspecting possible drug-induced thrombocytopenia. Furthermore, she was administered thrombopoietin receptor agonists in view of the possibility of idiopathic thrombocytopenic purpura, along with maximum support for right heart failure. Subsequently, her platelet count increased to >70,000/µL, enabling her to successfully undergo bilateral lung transplantation. Post-bilateral lung transplantation, pulmonary arterial hypertension as well as thrombocytopenia appeared to have resolved. In this case, we suspected that thrombocytopenia could have resulted owing to a combination of pulmonary arterial hypertension, right heart failure, drug interactions, and idiopathic thrombocytopenic purpura. Thrombocytopenia is a very critical condition in patients with pulmonary arterial hypertension, especially those awaiting lung transplantation. Several approaches are known to improve intractable thrombocytopenia in patients with pulmonary arterial hypertension.
尽管由于新型药物的出现，特发性肺动脉高压的预后有了显著改善，但肺移植仍然是重度肺动脉高压的关键治疗选择。因此，对于等待肺移植的患者来说，避免包括血小板减少症在内的并发症至关重要，因为这些并发症可能会影响他们的手术结果。在此，我们介绍一名21岁女性的病例，她在15岁时被诊断为特发性肺动脉高压。在等待肺移植期间，她出现了血小板减少症。怀疑可能是药物性血小板减少症，于是将她的药物从依前列醇换成了曲前列尼尔。此外，鉴于存在特发性血小板减少性紫癜的可能性，她接受了血小板生成素受体激动剂治疗，并对右心衰竭给予了最大程度的支持。随后，她的血小板计数升至>70,000/µL，使其能够成功接受双侧肺移植。双侧肺移植后，肺动脉高压以及血小板减少症似乎都得到了缓解。在这个病例中，我们怀疑血小板减少症可能是由肺动脉高压、右心衰竭、药物相互作用以及特发性血小板减少性紫癜共同导致的。血小板减少症在肺动脉高压患者中，尤其是在等待肺移植的患者中，是一种非常危急的情况。已知有几种方法可改善肺动脉高压患者难治性血小板减少症。
