Ischemic stroke as a presenting manifestation of polycythemia vera: a narrative review

Abstract

Abstract Polycythemia vera (PV) is a myeloproliferative disorder associated with an increased risk of cerebrovascular diseases. In this narrative review, we aimed to analyze the relationships between acute ischemic stroke and PV. We conducted a PubMed/Medline and Web of Sciences Database search using MeSH major terms. We found 75 articles and finally considered 12 case reports and 11 cohort studies. The ischemic stroke resulted as the first manifestation of PV in up to 16.2% of cases; the cumulative rate of cerebrovascular events was up to 5.5 per 100 persons per year and stroke accounted for 8.8% of all PV-related deaths; age, mutations, and a previous history of thrombosis were the main risk factors. The best approach to reduce stroke recurrence risk is unclear, even if some evidence suggests a potential role of lowering hematocrit below 45%. Ischemic stroke represents one of the most common PV manifestations but, despite their relationship, patients with both diseases have a very heterogeneous clinical course and management. PV-related strokes often remain underdiagnosed, especially for the low prevalence of PV. An early diagnosis could lead to prompt treatment with phlebotomy, cytoreduction, and low-dose aspirin to decrease the risk of recurrences. Clinicians should be aware of PV as a risk factor for stroke when approaching the differential diagnosis of cryptogenic strokes. An early diagnosis could positively influence patients’ management and clinical outcomes. Further studies are required to evaluate the role of PV treatments in the prevention of cerebrovascular disease.