第 5 组肺动脉高压 (PH) 涵盖多种疾病，其中少数病例将其与 T 细胞大颗粒淋巴细胞 (LGL) 白血病联系起来。我们报告了一名 76 岁女性的病例，她被诊断患有 LGL 白血病并伴有 PH，接受口服三重肺动脉高压 (PAH) 治疗。她最初表现为劳累时呼吸困难；评估显示严重的毛细血管前PH。使用环磷酰胺治疗白血病以及他达拉非和马西腾坦治疗 PH 可使症状和血流动力学持续改善超过 3 年。当时，PH值恶化促使添加selexipag，导致临床持续改善5年。该病例例证了 PAH 治疗对白血病相关 PH 具有持续益处的潜力，并强调需要继续研究 LGL 白血病与 PH 之间的机制关系，以期确定新的管理策略。版权所有 © 2024 美国胸科医师学会。由爱思唯尔公司出版。保留所有权利。

Group 5 pulmonary hypertension (PH) encompasses diverse diseases, with a few cases linking it to T-cell large granular lymphocytic (LGL) leukemia. We report a case of a 76-year-old woman, diagnosed with LGL leukemia and concomitant PH, treated with oral triple pulmonary arterial hypertension (PAH) therapy. She initially presented with dyspnea on exertion; evaluation revealed severe precapillary PH. Implementing cyclophosphamide for leukemia along with tadalafil and macitentan for PH led to sustained symptomatic and hemodynamic improvement for over 3 years. At that time, deterioration in PH prompted the addition of selexipag, resulting in sustained clinical improvement for an additional 5 years. This case exemplifies the potential for sustained benefits of PAH therapy in leukemia-associated PH and highlights the need for continued research on the mechanistic relationship between LGL leukemia and PH, with the hope of identifying new management strategies.Copyright © 2024 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.