Jiao Lidong, Huang Xiaoqin, Fan Chunqiu, Zhao Hong, Li Zhen, Shen Huixin, Chen Jian, Duan Jiangang
Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, People's Republic of China.
Department of Hematology, Xuanwu Hospital, Capital Medical University, Beijing, People's Republic of China.
Neuropsychiatr Dis Treat. 2021 Apr 22;17:1195-1206. doi: 10.2147/NDT.S294712. eCollection 2021.
Essential thrombocythemia (ET) is a rare cause of cerebral venous sinus thrombosis (CVST). Analysis of the risk factors and treatment therapies of CVST in ET has yielded controversial findings.
We retrospectively investigated the clinical characteristics of CVST events in ET and compared baseline characteristics, causative factors, hematological effects, and treatments between ET patients with and without CVST.
Overall, 91 of 115 patients who met the ET diagnosis were included in this study. Among them, 23 (25.27%) patients met the diagnostic criteria of ET with CVST for inclusion, 14 (60.87%) of whom were females, with a median age of 34 (range 25-50). CVST diagnosis was made concomitantly to ET in 19 patients (82.61%). The most common symptom and sites of thrombosis of CVST was an acute or subacute headache and sigmoid sinuses, respectively. Compared with ET patients without CVST, ET patients with CVST were significantly younger (37.65±14.45 vs 60.93±13.46, <0.001) and had lower prevalence of hypertension (4.34 vs 32.35%, =0.003) and coronary artery disease (0 vs 14.71%, = 0.045). Patients with CVST presented with significant lower platelet count (510.39±176.71 vs 750.82±249.10, < 0.001) and higher score of IPSET-thrombosis (=0.017). Multivariate logistic regression analysis indicated that age (=0.002, OR 1.096, 95% CI 1.035-1.161), at least one CVRF ( = 0.024, OR 0.037, 95% CI 0.002-0.649), platelet count (=0.045, OR 0.994, 95% CI 0.989-1.001), and lower percentage of antiplatelet therapy (=0.035, OR 0.307, 95% CI 0.001-1.280) significantly contributed to the risk of CVST in ET.
Most patients (95.65%) had a favorable outcome without recurrence after standard anticoagulant and cytoreductive treatment at last follow-up. These findings indicate that CVST may be the initial presentation of ET, with its detection crucial for early diagnosis and appropriate management. Anticoagulant and cytoreductive therapies should be recommended for preventing ET-related CVST with V617F mutation.
原发性血小板增多症（ET）是脑静脉窦血栓形成（CVST）的罕见病因。对ET中CVST的危险因素及治疗方法的分析已产生了有争议的结果。
我们回顾性研究了ET中CVST事件的临床特征，并比较了有和没有CVST的ET患者之间的基线特征、致病因素、血液学影响及治疗情况。
总体而言，115例符合ET诊断的患者中有91例纳入本研究。其中，23例（25.27%）患者符合ET合并CVST的诊断标准而被纳入，其中14例（60.87%）为女性，中位年龄为34岁（范围25 - 50岁）。19例患者（82.61%）在诊断ET的同时诊断出CVST。CVST最常见的症状和血栓形成部位分别是急性或亚急性头痛和乙状窦。与没有CVST的ET患者相比，有CVST的ET患者明显更年轻（37.65±14.45对60.93±13.46，<0.001），高血压患病率更低（4.34%对32.35%，=0.003），冠状动脉疾病患病率更低（0对14.71%，=0.045）。有CVST的患者血小板计数显著更低（510.39±176.71对750.82±249.10，<0.001），IPSET - 血栓形成评分更高（=0.017）。多因素逻辑回归分析表明，年龄（=0.002，OR 1.096，95%CI 1.035 - 1.161）、至少一种心血管危险因素（=0.024，OR 0.037，95%CI 0.002 - 0.649）、血小板计数（=0.045，OR 0.994，95%CI 0.989 - 1.001）以及抗血小板治疗的较低比例（=0.035，OR 0.307，95%CI 0.001 - 1.280）对ET中CVST的风险有显著影响。
在最后一次随访时，大多数患者（95.65%）在接受标准抗凝和细胞减灭治疗后预后良好且无复发。这些发现表明CVST可能是ET的首发表现，其检测对于早期诊断和恰当管理至关重要。对于预防具有V617F突变的ET相关CVST，应推荐抗凝和细胞减灭治疗。
