Study of Pulmonary Hypertension in Patients with Primary Myelofibrosis in Sohag University Hospital

Background: Pulmonary hypertension (PH) is a serious complex pathophysiological disorder that may be related to multiple clinical conditions and associated with high morbidity and mortality rates. It is a major complication of myeloproliferative neoplasms (MPNs) including primary myelofibrosis (PMF) mainly in advanced disease. Development of PH in PMF patients has a bad impact on prognosis of the disease and on the survival in those patients. PH prevalence in PMF and predictors for the disorder are not well established. Objective: The main objective of this thesis is to determine the prevalence of pulmonary hypertension in patients with primary myelofibrosis and to find different parameters that can predict PH in those patients.Results: Our results revealed that 13 patients out of 28 studied PMF patients had PH. There was a significant relationship between old age, long PMF duration since diagnosis at time of evaluation, presence of thrombotic events mainly portal vein thrombosis, anemia, and hyperuricemia and occurrence of PH. The two groups (PH & Non-PH groups) did not differ significantly in terms of gender, comorbidities, WBCs count, platelets count, serum lactate dehydrogenase level, and splenic diameter measured by abdominal ultrasound, presence of JAK 2 & Calreticulin (CALR) gene mutation.Conclusion: PH prevalence in PMF patients in our study was 46.4%. Our findings suggest that old age, long PMF duration since diagnosis, and hyperuricemia appear to be valuable for predicting the development of PH in those patients.