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Left Atrial Stenosis Induced Pulmonary Venous Arterialization and Group 2 Pulmonary Hypertension in Rat
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Pulmonary extra-medullary hematopoiesis and pulmonary hypertension from underlying polycythemia vera: a case series

Inderjit Singh1, Geoffrey Mikita2, Daniel Green3

  • 1Division of Pulmonary/Critical Care Medicine, Brigham and Women's Hospital, Boston, MA, USA.

Pulmonary Circulation
|July 7, 2017
PubMed

Insights

Pulmonary hypertension in myeloproliferative neoplasia is linked to extra-medullary hematopoiesis. This case series details pulmonary EMH and PH in polycythemia vera patients.

Area of Science:

  • Hematology
  • Pulmonary Medicine
  • Oncology

Background:

  • Myeloproliferative neoplasms (MPNs) are a group of clonal hematopoietic stem cell disorders.
  • Pulmonary hypertension (PH) associated with MPNs is classified under Group 5.
  • The Janus Kinase 2 (JAK2) mutation, p.V617F, is implicated in several MPNs, including polycythemia vera.

Observation:

  • Pulmonary extra-medullary hematopoiesis (EMH) is the presence of hematopoietic precursor cells in the lungs.
  • EMH is a rare complication, particularly associated with myelofibrosis.
  • This series focuses on pulmonary EMH and PH in the context of polycythemia vera.

Findings:

  • The study highlights the clinical, pathological, and radiological characteristics of pulmonary EMH.
  • It examines the association between polycythemia vera and the development of PH.
  • Case presentations illustrate the multifaceted presentation of this rare complication.

Implications:

  • Understanding the link between MPNs and PH can improve diagnostic strategies.
  • Recognizing pulmonary EMH as a complication of polycythemia vera may guide treatment.
  • Further research into the mechanisms of MPN-associated PH is warranted.
Keywords:
myelofibrosispulmonary arterial hypertensionpulmonary extra-medullary hematopoiesis

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