CLINICAL AND FUNCTIONAL STATE OF BRONCHOPULMONARY SYSTEM IN PATIENTS WITH IDIOPATHIC MYELOFIBROSIS

Abstract

Clinical and functional state of bronchopulmonary system in 24 patients with chronic idiopathic myelofibrosis was studied. Bronchoscopy with endobronchial laser Doppler flowmetry, biopsy of bronchi mucosa, complex ultrasound study of the heart and functional state of diaphragm were done to all patients. At idiopathic myelofibrosis the disorder of endobronchial microhemocirculation was diagnosed, important causes of which are anemic syndrome, thrombocytosis, endothelium dysfunction, and hyperleukocytosis for the development of blast crisis. A consequence of the observed changes in the microcirculation in idiopathic myelofibrosis is a disorder of tissue trophism, tissue hypoxia, local metabolic disorder, resulting in developing atrophic changes in bronchial mucosa. The decrease of diaphragm contractility in the patients with idiopathic myelofibrosis is conditioned by its compression by significantly enlarged lien and liver, and in the stage of blast crisis by specific leukemic damage (leukemic infiltration, atrophic changes, hemorrhage, leukostasis in vessels). Under progression of idiopathic myelofibrosis there are disorders of systolic and diastolic function of right and left ventricles. At the late stages of neoplastic progression of idiopathic myelofibrosis hypoxemia and pulmonary hypertension develop.