• Daniel J Strick, Harrison W Farber, Nicholas S Hill, Ioana R Preston, Natasha M Pradhan, and Bipin Malla.
• Tufts Medical Center, Boston, MA. Electronic address: daniel.strick@tuftsmedicine.org.
• Chest. 2024 Jul 1; 166 (1): e1e3e1-e3.

AbstractGroup 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.

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