A Difficult Case in Clinical Practice: Combined Polycythaemia Vera and Protein S Deficiency in a Patient with both Thrombotic and Bleeding Complications OD11-OD14
Dr. Ashwal Adamane Jayaram,
Associate Professor, Department of Cardilogy, Kasturba Medical College, Manipal Academy of Higher Education, Manipal-576104, Karnataka, India.
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.