Cerebral venous sinus thrombosis in JAK2 V617F positive polycythaemia vera presenting as a haemorrhagic stroke

Abstract

Background: Cerebral venous sinus thrombosis (CVST) has been reported to present with intracranial haemorrhage (ICH) due to its varying effects on cerebral circulation. Polycythaemia rubra vera (PRV) is one of the rare causes of CVST. Case Report: We present a 53-year-old gentleman with vascular dementia who presented with right upper and lower limb weakness. His NCCT brain revealed an ICH involving the left internal capsule. His FBC revealed polycythaemia. A cerebral CT venogram revealed thrombosis of cerebral venous sinuses. JAK-2 V617F mutation was detected confirming primary polycythaemia which was treated with antithrombotic and cytoreductive therapy. Discussion: This is a rare case of PRV presenting with CVST as the initial presentation. It is important to recognize the underlying cause which requires specific management to prevent future recurrences.

Keywords

• cerebral venous sinus thrombosis
• polycythaemia rubra vera
• jak-2 v617f mutation