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

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...

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Autores principales: Singh, Inderjit, Mikita, Geoffrey, Green, Daniel, Risquez, Cristobal, Sanders, Abraham
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2017
Materias:
Case Reports
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5448544/
https://www.ncbi.nlm.nih.gov/pubmed/28680586
http://dx.doi.org/10.1177/2045893217702064
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author Singh, Inderjit
Mikita, Geoffrey
Green, Daniel
Risquez, Cristobal
Sanders, Abraham
author_facet Singh, Inderjit
Mikita, Geoffrey
Green, Daniel
Risquez, Cristobal
Sanders, Abraham
author_sort Singh, Inderjit
collection PubMed
description 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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spelling pubmed-54485442017-06-08 Pulmonary extra-medullary hematopoiesis and pulmonary hypertension from underlying polycythemia vera: a case series Singh, Inderjit Mikita, Geoffrey Green, Daniel Risquez, Cristobal Sanders, Abraham Pulm Circ Case Reports 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. SAGE Publications 2017-03-10 /pmc/articles/PMC5448544/ /pubmed/28680586 http://dx.doi.org/10.1177/2045893217702064 Text en © 2017 by Pulmonary Vascular Research Institute http://creativecommons.org/licenses/by-nc/3.0/ This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 3.0 License (http://www.creativecommons.org/licenses/by-nc/3.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Case Reports
Singh, Inderjit
Mikita, Geoffrey
Green, Daniel
Risquez, Cristobal
Sanders, Abraham
Pulmonary extra-medullary hematopoiesis and pulmonary hypertension from underlying polycythemia vera: a case series
title Pulmonary extra-medullary hematopoiesis and pulmonary hypertension from underlying polycythemia vera: a case series
title_full Pulmonary extra-medullary hematopoiesis and pulmonary hypertension from underlying polycythemia vera: a case series
title_fullStr Pulmonary extra-medullary hematopoiesis and pulmonary hypertension from underlying polycythemia vera: a case series
title_full_unstemmed Pulmonary extra-medullary hematopoiesis and pulmonary hypertension from underlying polycythemia vera: a case series
title_short Pulmonary extra-medullary hematopoiesis and pulmonary hypertension from underlying polycythemia vera: a case series
title_sort pulmonary extra-medullary hematopoiesis and pulmonary hypertension from underlying polycythemia vera: a case series
topic Case Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5448544/
https://www.ncbi.nlm.nih.gov/pubmed/28680586
http://dx.doi.org/10.1177/2045893217702064
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