Pulmonary extra-medullary hematopoiesis and pulmonary hypertension from underlying polycythemia vera: a case series
نویسندگان
چکیده
Myeloproliferative neoplasia (MPN)-associated pulmonary hypertension (PH) is included in group five of the most recent clinical classification of PH.1 The MPNs are a heterogeneous group of disorders that includes disorders with primary expression of a myeloid phenotype and disorders characterized by expression of the Janus Kinase 2 (JAK2) mutation, p.V617F. The latter includes essential thrombocytosis, polycythemia vera, and idiopathic myelofibrosis.2 Pulmonary extra-medullary hematopoiesis (EMH) refers to the presence of hematopoietic precursor cells in the lung. It is a rare complication associated with myelofibrosis. Here we present a case series highlighting the clinical-pathological-radiological features of pulmonary EMH and PH from underlying polycythemia vera.
منابع مشابه
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 di...
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