Pulmonary hypertension in a patient with polycythemia vera: a case report

Polycythemia vera (PV) is an insidious and progressive myeloproliferative neoplasm (MPN) characterized by an increase in red blood cell mass, which is almost always associated with a mutation in the Janus kinase 2 (JAK2) 2 gene. It can be complicated by pulmonary hypertension (PH). PH due to MPN is classified in WHO group 5: PH due to various chronic diseases with poorly characterized or understood mechanisms. Furthermore, the mechanisms of PH can hemodynamically differ between precapillary, postcapillary, and combined postcapillary and precapillary PH. We describe a case of combined pre- and post-postcapillary PH in a patient with newly diagnosed PV. Due to the diverse pathways of PH in PV, such as chro­nic thromboembolic PH, pulmonary arteriopathy, and left-sided heart dise­ase, the ideal therapeutic approach for patients with PH and PV has not been established. Case reports have demonstrated that JAK inhibition with ruxolitinib improved MPN-associated PH, leading to the initiation of this therapy in our patient.