Pulmonary Hypertension Associated with Pulmonary Veno-occlusive Disease in Patients with Polycythemia Vera

A 65-year-old man was diagnosed with polycythemia vera (PV) and treated with hydroxyurea. Three years later, he was admitted to our institution for severe hypoxia. Right heart catheterization revealed that the patient had pulmonary hypertension (PH). In addition, radiographic findings and resistance to pulmonary vasodilators led to the diagnosis of PH associated with pulmonary veno-occlusive disease. The administration of ruxolitinib improved his hematopoiesis and respiratory failure. While the disease is relatively common in Europe and the United States, limited data exist regarding myeloproliferative neoplasm complicated with PH in Japan. PH should be considered a potential complication and screened during the clinical care of patients with myeloproliferative neoplasms.