1.
Rev Med Interne
; 30(9): 803-5, 2009 Sep.
Article
in French
| MEDLINE
| ID: mdl-19674818
ABSTRACT
There are several possible pathophysiological links between the development of pulmonary hypertension and myelofibrosis with myeloid metaplasia. We report a woman with myelofibrosis and myeloid metaplasia who presented with dyspnea and massive, painful splenomegaly. Right heart catheterization evidenced pulmonary hypertension. Her management consisted of splenic irradiation associated to sildenafil. Dyspnea in patients with myelofibrosis and myeloid metaplasia can be secondary to pulmonary hypertension and conversely the differential diagnosis of pulmonary hypertension should include a myeloproliferative syndrome.