Pulmonary hypertension in patients with myelofibrosis secondary to myeloproliferative diseases

We examined the clinical characteristics of six patients with myelofibrosis secondary to myeloproliferative diseases whose clinical courses were compl icated by pulmonary hypertension to determine possible causal links between the two disorders. Six patients (four male, two female), with diagnoses of myeloproliferative disease, myelofibrosis (one with polycythemia vera, thr ee with agnogenic myeloid metaplasia, one with unclassified myeloproliferat ive syndrome, one with essential thrombocytosis), and pulmonary hypertensio n are presented. Measurement of the pulmonary artery pressure was performed by Doppler echocardiography in all patients and by right sided heart cathe terization in four patients. The range of resting pulmonary artery systolic pressure was 35 to 47 mmHg above the mean right atrium by echocardiography . One patient had autopsy evidence of pulmonary myeloid metaplasia and inte rstitial fibrosis; another had acute leukemic infiltration of the lung pare nchyma. All patients had thrombocytosis; symptomatology in one patient with marked thrombocytosis improved with plateletpheresis. Two patients suffere d systemic thrombosis. All patients had severe hepatomegaly. Two patients h ad evidence of left ventricular dysfunction. The interval between the devel opment of dyspnea and death was less than seven months in five of the patie nts. A causal link between pulmonary hypertension and myelofibrosis seconda ry to myeloproliferative diseases is suggested for each patient. Hematopoie tic infiltration of the pulmonary parenchyma, portal hypertension, thromboc ytosis, hypercoagulability, and left ventricular failure may account in par t for the development of pulmonary hypertension in these patients. Patients with myelofibrosis and dyspnea should have Doppler echocardiography to eva luate pulmonary artery pressures. (C) 1999 Wiley-Liss, Inc.