A Difficult Case in Clinical Practice: Combined Polycythaemia Vera and Protein S Deficiency in a Patient with both Thrombotic and Bleeding Complications
Published: April 1, 2018 | DOI: https://doi.org/10.7860/JCDR/2018/31717.11438
Sushanth Wattal, Ashwal Adamane Jayaram, Padmakumar Ramachandran, Abdul UK Razak, Suheil Dhanse
1. Senior Resident, Department of Cardiology, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, Karnataka, India.
2. Associate Professor, Department of Cardiology, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, Karnataka, India.
3. Professor, Department of Cardiology, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, Karnataka, India.
4. Assistant Professor, Department of Cardiology, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, Karnataka, India.
5. Senior Resident, Department of Cardiology, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, Karnataka, India.
Correspondence
Dr. Ashwal Adamane Jayaram,
Associate Professor, Department of Cardilogy, Kasturba Medical College, Manipal Academy of Higher Education, Manipal-576104, Karnataka, India.
E-mail: dr.ashwal@gmail.com
Polycythemia Vera (PV) is a chronic myeloproliferative disorder which is characterised by a high risk of developing arterial as well as venous thromboembolic complications like deep vein thrombosis, stroke, cortical vein thrombosis and rarely acute Myocardial Infarction (MI). PV has a risk rate of major thrombosis as high as 50%. Bleeding, thrombotic, and vascular complications are the major causes of morbidity and mortality, occurring in 40 to 60% of these patients. MI with heart failure is the most common cause of death in these patients. Congenital Protein S deficiency is an autosomal dominant disease. Venous thrombosis develops in 60-80% of patients who are heterozygous for Protein S deficiency. Hereditary Protein S deficiency is a well known risk factor not only for venous thrombosis but also for arterial thrombosis including MI and stroke. However, association of Protein S deficiency with arterial thrombosis is less appreciated. Very rarely, we find all the varied thrombotic complications in one patient. We hereby present a case who had varied thrombotic presentations due to underlying polycythemia with Protein S deficiency.
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