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Article Abstract
Background: Pulmonary hypertension (PH) has rarely been associated with myeloproliferative neoplasms (MPNs), classified as World Health Organization group 5 PH. Progression of MPN may result in acute myelogenous leukemia (AML).
Case Summary: We describe a young woman with AML arising from MPN presenting with PH. Both the AML and PH were alleviated with pulmonary vasodilators and chemotherapy. The patient eventually underwent allogeneic hematopoietic stem cell transplantation, achieving remission. Several months later, she presented with respiratory symptoms and worsening of PH, signaling the relapse of her AML, subsequently confirmed with right heart catheterization and bone marrow biopsies.
Discussion: Our case highlights the interplay between PH and hematologic malignancies. Literature suggests that PH progression may parallel the progression of MPN.
Take-home Messages: Prompt recognition of PH in those with MPNs is needed to facilitate appropriate interventions in a multidisciplinary manner. Although an association between PH and MPN exists, further studies into the underlying mechanisms and their interplay is needed.
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| http://dx.doi.org/10.1016/j.jaccas.2025.104136 | DOI Listing |
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