Cases of ischemic stroke associated with Ph-negative myeloproliferative diseases

Myeloproliferative neoplasms (MPN) are clonal diseases that arise at the hematopoietic stem cell level, characterized by the proliferation of one or more myelopoietic cell lines in the bone marrow with features of preserved terminal differentiation. According to the fifth edition of the World Health Organization (WHO) classification (2022), Ph-negative MPNs include polycythemia vera (PV), essential thrombocythemia (ET), primary myelofibrosis (PMF), chronic neut23rophilic leukemia, chronic eosinophilic leukemia, juvenile myelomonocytic leukemia, and myeloproliferative neoplasm, unclassified (MPN-U). ET, PV, PMF, and MPN-U have similar genetic characteristics and account for 98 % of all Ph-negative MPNs. This article describes three clinical cases of patients with ischemic stroke (IS) and a verified diagnosis of Ph-negative myeloproliferative neoplasm (one clinical cases of IS against the background of polycythemia vera, two against the background of essential thrombocythemia), admitted to the Regional Vascular Center, Republic Hospital No. 2 – Center for Emergency Medical Aid. A literature review is presented, risk factors and possible pathways for the development of venous and arterial thrombosis in MPN are considered, as well as the role of the JAK2 V617F mutation in the development of cerebrovascular complications. It is shown that, despite their rarity, MPNs are a significant cause of stroke, with IS often the first manifestation of MPN. Heterogeneity of vascular lesions in IS against the background of MPN, as well as a high probability of developing recurrent cerebral infarctions, are demonstrated. The issue of the need for timely diagnosis of MPN, cytoreductive therapy along with antithrombotic therapy for the prevention of thrombotic complications is considered separately.

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