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

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.