Pulmonary extra-medullary hematopoiesis and pulmonary hypertension from underlying polycythemia vera: a case series. uri icon

Overview

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.

publication date

  • March 10, 2017

Identity

PubMed Central ID

  • PMC4849178

Scopus Document Identifier

  • 85032000996

Digital Object Identifier (DOI)

  • 10.1177/2045893217702064

PubMed ID

  • 28680586

Additional Document Info

volume

  • 7

issue

  • 1