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

PVRI Member Authors: Inderjit Singh

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.

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Topics

Pulmonary Arterial Hypertension

Authors

Inderjit Singh, Geoffrey Mikita, Daniel Green, Cristobal Risquez, Abraham Sanders

Published in:

Pulmonary Circulation Vol 7: No 1 cover image

March 2017

Pulmonary Circulation Vol 7: No 1

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