Pulmonary extra-medullary hematopoiesis and pulmonary hypertension from underlying polycythemia vera: a case series. (March 2017)

• Record Type: Journal Article
• Authors: Singh, Inderjit; Mikita, Geoffrey; Green, Daniel; Risquez, Cristobal; Sanders, Abraham
• Is Part Of: Pulmonary circulation. Volume 7:Number 1(2017)
• Page Start: 261
• Page End: 267
• Publication Date: 2017-03
• Subjects: pulmonary arterial hypertension -- pulmonary extra-medullary hematopoiesis -- myelofibrosis; Pulmonary circulation -- Periodicals; Pulmonary circulation; Electronic journals -- Sciences; Periodicals; 616.24005
• DOI: 10.1177/2045893217702064
• Languages: English
• ISSNs: 2045-8932

Abstract

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.