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Pulmonary hypertension as a risk factor in JAK2-positive polycythemia rubra vera
Shaimaa A Ahmed1, Alaa Rashad2, Asmaa Nafady MD 3, Asmaa Y Shazly4, Areej Alkhateeb5, Mohamed A Elsenbesya6
1 Haematology Unit, Department of Internal Medicine, Faculty of Medicine, South Valley University, Qena, Egypt
2 Department of Chest Diseases and Tuberculosis, Faculty of Medicine, South Valley University, Qena, Egypt
3 Department of Clinical Pathology, Faculty of Medicine, Assiut University, Assiut; Department of Clinical and Chemical Pathology, Qena Faculty of Medicine, South Valley University, Qena, Egypt
4 Department of Internal Medicine, Faculty of Medicine, South Valley University, Qena, Egypt
5 Cardiology Division, Department of Internal Medicine, Faculty of Medicine, South Valley University, Qena, Egypt
6 Department of Internal Medicine, Faculty of Medicine, South Valley University, Qena; Department of Internal Medicine, Arabian Gulf University (AGU), Bahrain, Egypt
Correspondence Address:
Asmaa Nafady
Clinical and Chemical Pathology, Qena Faculty of Medicine, South Valley University, Qena, Department of Clinical Pathology, Faculty of Medicine, Assiut University, Assiut, 83523
Egypt
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/ejh.ejh_9_21
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Introduction The occurrence of primary pulmonary arterial hypertension (PAH (in primary myeloid proliferation neoplasms seems quite high, up to 22% in polycythemia vera. Polycythemia rubra vera (PRV) is one of the myeloproliferative neoplasms. We aimed to study the incidence of PAH among patients with PRV.
Patients and methods A prospective cross-section study was performed on 60 (PRV) patients with PRV confirmed by bone marrow and JAK2 positivity. Abdominal ultrasonography, transthoracic echocardiography, and computed tomography chest were done to estimate the pulmonary hypertension (HTN) and exclude other chest diseases.
Results Among the studied 60 patients, we found 14 patients with pulmonary HTN (23.4%) who had significantly increased incidence of comorbidities than patients with normal pulmonary pressure (P=0.009). Moreover, there were statistically significant differences in the size of spleen by ultrasound between the two groups (P=0.008). Patients with pulmonary HTN had a higher hemoglobin level compared with those with normal pulmonary pressure (P=0.006). There was a significant positive correlation between pulmonary pressure and existing comorbidities (diabetes mellitus, HTN, or both) but no correlation with the size of the spleen, hemoglobin level, or white blood cells.
Conclusion The prevalence of PAH in JAK2-positive patients with PRV is 76.6%, and there was significant relationship between hematological parameters (hemoglobin, white blood cells, lactate dehydrogenase, and urea level and PAH in JAK2-positive patients with PRV.
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