Polycythemia Vera Causing Cerebrovascular Thrombosis: A Rare Presentation of Stroke in a Young Patient
Objective:
We describe a rare case of a young patient who presented with acute ischemic strokes as the initial presentation of polycythemia vera.
Background:
Overall, 10-15% of adult stroke patients are younger than 50 and only 1% of strokes are later attributed to hematologic etiologies. Seen predominately in older patients, polycythemia vera (PV), a chronic myeloproliferative disease, is rarely diagnosed in patients under 40 (only 4-7%). However, ~70% of all thrombotic events at presentation in PV are cerebrovascular in nature.
Results:
A previously healthy 23-year-old African American male presented outside of the window for acute stroke interventions with 2 days of left-sided hemiparesis. Non-contrasted computed tomography (CT) imaging revealed hypodensities in the right frontal and parietal lobes. CT angiography demonstrated a right, proximal middle cerebral artery (MCA) occlusion and basilar artery stenosis in addition to generally diminutive posterior arterial vasculature. Magnetic resonance imaging confirmed multifocal areas of restricted diffusion involving bilateral MCA territories evident of acute infarctions. Aspirin monotherapy and a high-intensity-statin were first initiated. Other than a hemoglobin of 18 g/dL, initial serum laboratory and cardiac imaging studies were unrevealing. Cerebrospinal fluid analysis demonstrated a mild lymphocytic pleocytosis, a normal protein, and negative infectious studies. Formal cerebral arteriogram was normal without findings of vasculitis. Hematology was consulted and the patient was found to have a positive JAK2 V617F mutation with a normal erythropoietin level indicative of primary PV. The patient was then started on Hydroxyurea, aggressive hydration and underwent phlebotomy for further secondary stroke prevention prior to discharge to rehabilitation facility.
Conclusions:
Cerebrovascular thrombosis at presentation in PV is common, however, PV is uncommon in young patients. In this rare case, mildly elevated hemoglobin was the only finding that eventually led to accurate diagnosis and treatment.