Kuznetsova Polina I, Raskurazhev Anton A, Konovalov Rodion N, Krotenkova Marina V, Chechetkin Andrey O, Lagoda Olga V, Melikhyan Anait L, Tanashyan Marine M
Department of Angioneurology, Research Center of Neurology, 125367 Moscow, Russia.
Neuroimaging Department, Research Center of Neurology, 125367 Moscow, Russia.
J Clin Med. 2021 Dec 21;11(1):13. doi: 10.3390/jcm11010013.
Philadelphia chromosome-negative myeloproliferative disorders (Ph-negative MPD) are a rare group of hematological diseases, including three distinct pathologies: essential thrombocythemia (ET), polycythemia vera (PV), and primary myelofibrosis (PMF). They most often manifest with thrombotic complications, including cerebrovascular events. Covert brain infarcts (CBIs) are defin ed as predominantly small ischemic cerebral lesions that are detected using magnetic resonance imaging (MRI) in the absence of clinical stroke events. The relationship between MPD and CBIs remains unclear.
Included in the study were 103 patients with the diagnosis of Ph-MPD (according to WHO 2016 criteria) (median age-47 (35; 54) years; 67% female). In total, 38 patients had ET, 42 had PV, and 23 had PMF. They underwent clinical examination, routine laboratory analyses (complete blood count), brain MRI, ultrasound carotid artery, flow-mediated dilatation (as a measure of endothelial dysfunction-FMD).
Overall, 23 patients experienced an ischemic stroke (as per MRI and/or clinical history), of which 16 (15.5%) could be classified as CBIs. The rate of CBIs per MPD subtype was statistically non-significant between groups ( = 0.35): ET-13.2%, PV-21.4%, and PMF-8.7%. The major vascular risk factors, including arterial hypertension, carotid atherosclerosis, and prior venous thrombosis, were not associated with CBIs ( > 0.05). Age was significantly higher in patients with CBIs compared to patients without MRI ischemic lesions: 50 (43; 57) years vs. 36 (29; 48) ( = 0.002). The frequency of headaches was comparable between the two groups. CBIs were associated with endothelial dysfunction (OR - 0.71 (95% CI: 0.49-0.90; = 0.02)) and higher hemoglobin levels (OR-1.21 (95% CI: 1.06-1.55); =0.03).
CBIs are common in patients with Ph-negative MPD. Arterial hypertension and carotid atherosclerosis were not associated with CBIs in this group of patients. The most significant factors in the development of CBIs were endothelial dysfunction (as measured by FMD) and high hemoglobin levels. Patients with Ph-negative MPD and CBIs were older and had more prevalent endothelial dysfunction.
费城染色体阴性骨髓增殖性疾病（Ph阴性MPD）是一组罕见的血液系统疾病，包括三种不同的病理类型：原发性血小板增多症（ET）、真性红细胞增多症（PV）和原发性骨髓纤维化（PMF）。它们最常表现为血栓形成并发症，包括脑血管事件。隐匿性脑梗死（CBI）被定义为主要是小的缺血性脑病变，在无临床中风事件的情况下通过磁共振成像（MRI）检测到。MPD与CBI之间的关系仍不清楚。
该研究纳入了103例诊断为Ph-MPD的患者（根据WHO 2016标准）（中位年龄47（35；54）岁；67%为女性）。其中，38例患有ET，42例患有PV，23例患有PMF。他们接受了临床检查、常规实验室分析（全血细胞计数）、脑部MRI、颈动脉超声、血流介导的血管舒张功能检测（作为内皮功能障碍的指标-FMD）。
总体而言，23例患者发生了缺血性中风（根据MRI和/或临床病史），其中16例（15.5%）可归类为CBI。各MPD亚型的CBI发生率在组间无统计学差异（P = 0.35）：ET为13.2%，PV为21.4%，PMF为8.7%。主要血管危险因素，包括动脉高血压、颈动脉粥样硬化和既往静脉血栓形成，与CBI无关（P>0.05）。与无MRI缺血性病变的患者相比，CBI患者的年龄显著更高：50（43；57）岁对36（29；48）岁（P = 0.002）。两组头痛频率相当。CBI与内皮功能障碍相关（OR - 0.71（95%CI：0.49 - 0.90；P = 0.02））和较高的血红蛋白水平相关（OR - 1.21（95%CI：1.06 - 1.55）；P = 0.03）。
CBI在Ph阴性MPD患者中很常见。在这组患者中，动脉高血压和颈动脉粥样硬化与CBI无关。CBI发生的最重要因素是内皮功能障碍（通过FMD测量）和高血红蛋白水平。Ph阴性MPD和CBI患者年龄更大，内皮功能障碍更普遍。
