Open AccessPulmonary extra‐medullary hematopoiesis and pulmonary hypertension from underlying polycythemia vera: a case series
Author(s) -
Singh Inderjit,
Mikita Geoffrey,
Green Daniel,
Risquez Cristobal,
Sanders Abraham
Publication year - 2017
Publication title -
pulmonary circulation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.791
H-Index - 40
ISSN - 2045-8940
DOI - 10.1177/2045893217702064
Subject(s) - medicine , pulmonary hypertension , myelofibrosis , polycythemia vera , extramedullary hematopoiesis , thrombocytosis , janus kinase 2 , haematopoiesis , pathological , pathology , myeloproliferative neoplasm , lung , myeloid , gastroenterology , stem cell , bone marrow , platelet , receptor , biology , genetics
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.