Pulmonary extra-medullary hematopoiesis and pulmonary hypertension from underlying polycythemia vera: A case series Academic Article uri icon

Overview

MeSH Major

  • Lung
  • Pulmonary Diffusing Capacity
  • Pulmonary Disease, Chronic Obstructive
  • Pulmonary Emphysema
  • Smoking

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

  • January 2017

Research

keywords

  • Academic Article

Identity

Language

  • eng

PubMed Central ID

  • PMC5448544

Digital Object Identifier (DOI)

  • 10.1177/2045893217702064

PubMed ID

  • 28680586

Additional Document Info

start page

  • 261

end page

  • 267

volume

  • 7

number

  • 1