异基因造血干细胞移植后骨髓纤维化相关肺动脉高压的缓解
Resolution of myelofibrosis-associated pulmonary arterial hypertension following allogeneic hematopoietic stem cell transplantation.
作者信息
Faiz Saadia A, Iliescu Cezar, Lopez-Mattei Juan, Patel Bela, Bashoura Lara, Popat Uday
机构信息
Department of Pulmonary Medicine, University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
Department of Cardiology, University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
出版信息
Pulm Circ. 2016 Dec;6(4):611-613. doi: 10.1086/687291.
We present the case of a 62-year-old man with myelofibrosis-associated pulmonary arterial hypertension (PAH) who underwent allogeneic hematopoietic stem cell transplantation with subsequent resolution of disease and PAH. Right heart catheterization was used to guide PAH therapy before and after transplantation. Drug interactions, adverse effects, and renal insufficiency posed clinical challenges for the management of PAH-specific medications after transplantation. PAH improved soon after transplantation, and vasoactive medications were tapered off. Resolution of PAH was confirmed with repeat measurement of pulmonary hemodynamic characteristics. Although the etiology and pathophysiology for the resolution of PAH was unclear, the myelopulmonary pathophysiologic link was likely to have contributed. This is the first report describing resolution of myelofibrosis-associated PAH after allogeneic hematopoietic stem cell transplantation.
我们报告了一例62岁患有骨髓纤维化相关肺动脉高压（PAH）的男性患者，该患者接受了异基因造血干细胞移植，随后疾病和PAH均得到缓解。移植前后均使用右心导管检查来指导PAH治疗。药物相互作用、不良反应和肾功能不全给移植后PAH特异性药物的管理带来了临床挑战。移植后PAH很快得到改善，血管活性药物逐渐减量。通过重复测量肺血流动力学特征证实了PAH的缓解。尽管PAH缓解的病因和病理生理学尚不清楚，但骨髓-肺病理生理联系可能起到了作用。这是第一份描述异基因造血干细胞移植后骨髓纤维化相关PAH缓解的报告。
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