Abstract
Introduction Hypereosinophilic syndrome (HES) is defined as persistent eosinophilia (>1.5/µL) without an identifiable cause and end organ damage, which can include thromboembolism. Here we present a case of cerebral venous sinus thrombosis (CVST) secondary to idiopathic HES.
Case Presentation A 66-year-old woman presented with somnolence, headache, vomiting, and right hemiparesis. Examination demonstrated right sided pyramidal weakness with global aphasia. Nail beds splinter haemorrhages were noted.
Imaging confirmed extensive, though asymmetric, CVST, vasogenic oedema and areas of venous infarction. Investigations revealed a persistently elevated eosinophil count of 2.2 to 2.9/µL (NR 0–0.5/µL). Bone marrow biopsy showed normal trilineage haematopoiesis and marked eosinophilia (20%). Genetic analysis for causes of HES were negative (PDGFRA/B and FGFR1). Investigation for autoimmune, infectious, and allergic disorders related HES including whole body CT were all negative. A thrombophilia screen was normal. The patient was diagnosed with idiopathic HES, resulting in CVST.
She was commenced on therapeutic Enoxaparin and eventually transitioned to warfarin. However, she represented two weeks later with worsening symptoms. Imaging showed extension of stroke and CVST. Thrombectomy was unsuccessful. She therefore was commenced on a short course of oral prednisone to treat the underlying pro-thrombotic state related to HES, to good effect. She made a full recovery and remains well now at 7 months. Eosinophil count remains normal.
Conclusion HES is a rarely reported condition but has been associated with systemic thrombosis, including CVST. Clinicians should be aware of the link, as such patients may require immunotherapy in addition to anticoagulation.
