Clinical Characteristics of Cerebrovascular Pathology with Patients Suffering from Ph-Negative Myeloproliferative Disease

Abstract

Background: Disturbances of microcirculation play a significant role in the development and progression of both acute and chronic cerebrovascular diseases (CVD) and may be associated with different hemogram abnormalities. One of the reasons of the prothrombogenic state of the endothelium is the increase in the number of blood corpuscles leading to (non-Ph) myeloproliferative disorders (MPD) including essential thrombocythemia (ET), polycythemia vera (PV), and primary myelofibrosis (PM). Materials and Methods: The study included 167 patients: 102 patients with Ph-MPD and the control group comprising 65 patients with CVD. According to MPD subtype, the patients were divided into three groups: patients with ET (37%, n = 38, male/female 7/31, age 52 ± 7 years), those with PV (40%, n = 41, male/female 20/21, age 50 ± 6 years) and those with PM (23%, n = 23, male/female 5/18, age 54 ± 4 years). Results: In 79% (n = 81) of cases in the study group (with Ph-MPD), patients had chronic CVD, with the most frequently identified symptoms being asthenia (92%) and headache (72%). Headache in Ph-MPD patients was more frequently (86%) associated with PM, while in patients with PV and ET it was equally distributed (70%). Neurological symptoms in 53% of cases were associated with focal changes of the brain on MRI localized in the subcortical area of the frontal and parietal lobes. Twenty-one (21%) patients suffered an acute cerebrovascular accident, 8 of them had thrombotic occlusion of one of the internal carotid arteries leading to hemispheric infarcts. Endothelial function (as measured by flow-dependent dilation of the brachial artery) was severely impaired in all study groups (median 5% with normal cut-off at 10%), the lowest degree of vasodilator activity being specific for patients with a history of stroke (p = 0.011). Conclusion: Patients suffering from MPD had asymptomatic focal changes in the brain in the absence of concomitant vascular disease (hypertension, atherosclerotic vascular disease, heart rhythm disorders) in 50% of cases. MPD, while remaining un- or underdiagnosed, presents a major concern in the cerebrovascular setting. A large number of thrombotic strokes occurring in patients with ET underline the necessity of early diagnostics and preventive therapy in these patients.

<i>JAK2</i> V617F Mutation and Large Cerebral Artery Disease in Patients with Myeloproliferative Neoplasms

“…Kato et al reported that of 10 consecutive patients with ischaemic stroke with ET analysed in their study, 2 (20%) had LCAD-related stroke 4) . Tanashyan et al reported that 21% of the young (≤ 58 years old) patients with MPNs (21/102) in their study experienced acute cerebrovascular events, and 8 of them (38%) had thrombotic occlusion of one of the internal carotid arteries 12) . In their prospective study, Nagai et al identified large vessel involvement in 5 of 23 (22%) patients with MPNs with ischaemic stroke 3) .…”

Acute ischemic stroke in the setting of essential thrombocytemia (clinical cases)

“…Одним из основных клинических проявлений данных нозологических форм Ph-негативных МПЗ являются тромбозы (как артериальные, так и венозные) различной локализации, которые встречаются до 40% случаев [4]. В ряде исследований показана ассоциация цереброваскулярной патологии с МПЗ [5], причем важной особенностью нередко является развитие острых нарушений мозгового кровообращения (ОНМК) как дебюта -первого клинического проявления «асимптомного» МПЗ [6]. Возникновение ОНМК возможно также во время специфической циторедуктивной, антиагрегантной терапии основного гематологического заболевания.…”