Clinical analysis of seven cases of intracranial hypertension with essential thrombocythemia

Abstract

Objective To report the presentation, clinical course and prognosis of intracranial hypertension (IH) with essential thrombocythemia (ET). Methods Retrospective data analysis was conducted. Seven cases of IH with ET were reviewed between January 2010 and December 2016. The clinical features, and the relationship between IH and ET were analyzed. Results IH complicated with ET was more common among young and middle-aged women. Sub acute or chronic course was more common. IH was easily misdiagnosed as optic neuritis in the early stage. The median platelet count of seven patients was 559×109/L. Paroxysmal black haze (four cases) was the most common of the first symptom. The head MRI showed empty sella in six cases. CT venography/DSA showed venous sinus thrombosis in three cases. All the patients were given intracranial pressure lowering and other symptomatic treatment. Five cases were treated with anti ET. Results showed remission in six cases and death in one case. Conclusions In the early stage IH was often misdiagnosed. Female was more common than male in IH with ET. Dural sinus blockage was common. In addition to symptomatic treatment such as lowering intracranial pressure, the patients should also be treated with anti ET. Key words: Intracranial hypertension; Thrombocythemia, essential; Prognosis