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

Abstract

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.

Cardiovascular Disease in Myeloproliferative Neoplasms

“… 55 , 56 Other potential etiologies of pulmonary hypertension in patients with MPNs include pulmonary extramedullary hematopoiesis and pulmonary veno-occlusive disease. 51 , 57 , 58 Additionally, patients with MPNs have a heightened risk for pulmonary embolism and recurrent venous thromboembolism; thus, chronic thromboembolic pulmonary hypertension likely contributes to the prevalence of pulmonary hypertension in this patient population. 59 , 60 …”

Dyspnoea and diffuse pulmonary nodules in a patient with pulmonary veno-occlusive disease: a case report and literature review

“…6 In a previous study of PVOD, the patients’ age at onset showed a relatively wide range, and the disease was reported from birth to the age of 70 years. 7 PVOD affects both sexes, but the incidence of genetically related PVOD is slightly higher in young women. Most patients with sporadic PVOD are older men at the time of diagnosis.…”

Pulmonary hypertension in patients with Philadelphia-negative myeloproliferative neoplasms: a single-center retrospective analysis of 225 patients

“…Some overt causes of PH such as veno-occlusive disease, pulmonary thromboembolism, and extramedullary hematopoiesis in the lungs have been identified in some MPN patients [ 19 , 21 , 34 ]. However, the causative factors and mechanisms are not clear in most cases, although some experiments have suggested several underlying mechanisms.…”