Case Report
Volume 1 Issue 1 - 2017
Polycythernia Vera induced Chronic Thromboembolic Pulmonary Hypertension
Geng-Peng Lin MD*, Yi-Feng Luo MD, Yu-Biao GuoMD and Can-Mao Xie MD
Respiratory Department, The First Affiliated Hospital of Sun Yat-sen University, Institute of Respiratory Diseases of Sun Yat-Sen University, China
*Corresponding Author: Geng-Peng Lin, Respiratory Department, The First Affiliated Hospital of Sun Yat-sen University, Institute of Respiratory Diseases of Sun Yat-Sen University, China.
Received: May 18, 2017; Published: May 22, 2017
Case Report
A 47-year-old man presented with progressive dyspnea over a period of 4 years. On physical examination, central cyanosis and nail clubbing were present. Cardiac examination revealed left parasternal heave and P2 > A2 on auscultation. The hemoglobin level was 22.0g per liter. Arterial blood gas analysis showed a pH of 7.42, a PaO2 of 44 mmHg, and a PaCO2 of 38 mmHg when he was breathing ambient air. A transthoracic echocardiogram exhibited right ventricular enlargement, severe tricuspid regurgitation and an estimated pulmonaryartery pressure of 112 mmHg. Plain (Figure1A) and contrast-enhanced (Figure1B) CT scan of the chest showed eccentric thrombotic material within the pulmonary arteries, with scattered calcification in the thrombi (arrow). The pulmonary-to-aorta diameter ratio was over 3. Enlargement of pulmonary arteries were also present (Figure1C, arrow). Bone marrow study showed erythroid hyperplasia and a mutation of JAK2V617F (mutation: wild-type 30.2). The diagnose of polycythernia vera was established. Hydroxyurea and warfarin were prescribed. Six months after discharge, his physical status worsened gradually but he refused to receive surgery.
Chronic thromboembolic pulmonary hypertension (CTPH) is one of the most common causes of pulmonary hypertension (PH), according to the recent clinical classification of PH [1]. Myeloproliferative diseases, especially polycythaemia vera and essential thrombocytosis, have been reported to be related to the development of CTPH. Pulmonary endarterectomy could be the treatment of choice in selected patients, however, the prognosis remains poor [2].
The authors acknowledge financial support from the National Natural Science Foundation of China (81502492) and the PhD Start-up Fund of Natural Science Foundation of Guangdong Province (2014A030310167).
  1. Simonneau G., et al. “Updated clinical classification of pulmonary hypertension”. Journal of the American College of Cardiology 54.1 (2009): S43–S54.
  2. Adir Y., et al. “Pulmonary hypertension in patients with chronic myeloproliferative disorders”. European Respiratory Review 35.6 (2010): 1396-1406.
Citation: Geng-Peng Lin., et al. “Polycythernia Vera induced Chronic Thromboembolic Pulmonary Hypertension”. Pulmonary Research and Respiratory Care 1.1 (2017): 24-25.
Copyright: © 2017 Geng-Peng Lin., et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.