Open AccessPulmonary hypertension diagnosed by echocardiography during idiopathic myelofibrosis
Author(s) -
Claudio Perrone,
R. Cartolari,
Beatrice Lupi,
Sergio Morelli
Publication year - 2010
Publication title -
multidisciplinary respiratory medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.72
H-Index - 28
eISSN - 2049-6958
pISSN - 1828-695X
DOI - 10.4081/mrm.2010.553
Subject(s) - medicine , myelofibrosis , extramedullary hematopoiesis , pulmonary hypertension , portal hypertension , myeloproliferative disorders , metaplasia , gastroenterology , pathology , haematopoiesis , bone marrow , cirrhosis , stem cell , biology , genetics
Idiopathic myelofibrosis is a rare chronic myeloproliferative disease leading to extramedullary hematopoiesis (myeloid metaplasia) with splenomegaly. The liver and less frequently other organs including the lung can be involved, therefore portal hypertension is relatively common. Pulmonary hypertension (PH) is only occasionally reported, although recent studies have suggested an association between PH and myeloproliferative disorders. We present a case of PH diagnosis by echocardiography in a patient affected by idiopathic myelofibrosis with portal hypertension.