Clinical Characteristics and Management of Cerebral Venous Sinus Thrombosis in Patients with Essential Thrombocythemia

Lidong Jiao; Xiaoqin Huang; Chunqiu Fan; Hong Zhao; Zhen Li; Huixin Shen; Jian Chen; Jiangang Duan

doi:10.2147/NDT.S294712

Clinical Characteristics and Management of Cerebral Venous Sinus Thrombosis in Patients with Essential Thrombocythemia

Neuropsychiatr Dis Treat. 2021 Apr 22:17:1195-1206. doi: 10.2147/NDT.S294712. eCollection 2021.

Authors

Lidong Jiao¹, Xiaoqin Huang¹, Chunqiu Fan¹, Hong Zhao², Zhen Li³, Huixin Shen¹, Jian Chen⁴, Jiangang Duan⁵

Affiliations

¹ Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, People's Republic of China.
² Department of Hematology, Xuanwu Hospital, Capital Medical University, Beijing, People's Republic of China.
³ Department of Ophthalmology, Xuanwu Hospital, Capital Medical University, Beijing, People's Republic of China.
⁴ Department of Neurosurgery, Xuanwu Hospital, Capital Medical University, Beijing, People's Republic of China.
⁵ Department of Emergency, Xuanwu Hospital, Capital Medical University, Beijing, People's Republic of China.

Abstract

Background and objective: 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.

Subjects and methods: 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.

Results: 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, P<0.001) and had lower prevalence of hypertension (4.34 vs 32.35%, P=0.003) and coronary artery disease (0 vs 14.71%, P = 0.045). Patients with CVST presented with signiﬁcant lower platelet count (510.39±176.71 vs 750.82±249.10, P< 0.001) and higher score of IPSET-thrombosis (P=0.017). Multivariate logistic regression analysis indicated that age (P=0.002, OR 1.096, 95% CI 1.035-1.161), at least one CVRF (P = 0.024, OR 0.037, 95% CI 0.002-0.649), platelet count (P=0.045, OR 0.994, 95% CI 0.989-1.001), and lower percentage of antiplatelet therapy (P=0.035, OR 0.307, 95% CI 0.001-1.280) significantly contributed to the risk of CVST in ET.

Conclusion: 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 JAK2 V617F mutation.

Keywords: JAK2 V617F; anticoagulant therapy; cerebral venous thrombosis; cytoreductive therapy; essential thrombocythemia.

Grants and funding

The study was funded by the Fund of the Capital Medical University (17JL-02) and the National Key Research and Development Program of China (2016YFC1300600; 2016YFC0901004).