Bibliographic Details
| Title: |
Group 5 Pulmonary Hypertension Associated With T-Cell Large Granular Lymphocytic Leukemia: Hemodynamics and Treatment. |
| Authors: |
Strick, Daniel J., Farber, Harrison W., Hill, Nicholas S., Preston, Ioana R., Pradhan, Natasha M., Malla, Bipin |
| Source: |
CHEST; Jul2024, Vol. 166 Issue 1, pe1-e3, 3p |
| Subject Terms: |
LYMPHOCYTIC leukemia, PULMONARY arterial hypertension, T cells, HEMODYNAMICS, BRAIN natriuretic factor |
| Abstract: |
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. [ABSTRACT FROM AUTHOR] |
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| Database: |
Complementary Index |
