The recent association of cerebral venous thrombosis (CVT) with COVID-19 vaccinations prompted the current retrospective review of 74 cases of CVT (median age = 44 years, range 15–85; 61% females) associated with myeloproliferative neoplasms (MPNs), seen at the Mayo Clinic, Catholic University of Rome, and University of Florence, between 1991 and 2021. Disease-specific frequencies were 1.3% (39/2893), 1.2% (21/1811) and 0.2% (3/1888) for essential thrombocythemia, polycythemia vera and primary myelofibrosis, respectively. Cerebral venous thrombosis occurred either prior to (n = 20, 27%), at (n = 32, 44%) or after (n = 22) MPN diagnosis. A total of 72% of patients presented with headaches. Transverse (51%), sagittal (43%) and sigmoid sinuses (35%) were involved with central nervous system hemorrhage noted in 10 (14%) patients. In all, 91% of tested patients harbored JAK2V617F. An underlying thrombophilic condition was identified in 19 (31%) cases and history of thrombosis in 10 (14%). Treatment for CVT included systemic anticoagulation alone (n = 27) or in conjunction with aspirin (n = 24), cytoreductive therapy (n = 14), or both (n = 9). At a median follow-up of 5.1 years (range 0.1–28.6), recurrent CVT was documented in three (4%) patients while recurrent arterial and venous thromboses and major hemorrhage were recorded in 11%, 9% and 14%, respectively. Follow-up neurological assessment revealed headaches (n = 9), vision loss (n = 1) and cognitive impairment (n = 1). The current study lends clarity to MPN-associated CVT and highlights its close association with JAK2V617F, younger age and female gender. Clinical features that distinguish COVID vaccine-related CVT from MPN-associated CVT include, in the latter, lower likelihood of concurrent venous thromboses and intracerebral hemorrhage; as a result, MPN-associated CVT was not fatal.

中文翻译：

---

脑静脉血栓形成和骨髓增生性肿瘤：74 例连续病例的三中心研究

最近脑静脉血栓形成 (CVT) 与 COVID-19 疫苗接种的关联促使目前对 74 例与骨髓增生性肿瘤 (MPN) 相关的 CVT（中位年龄 = 44 岁，范围 15-85；61% 女性）进行回顾性审查，见1991 年至 2021 年间，在罗马天主教大学和佛罗伦萨大学的梅奥诊所。疾病特异性频率分别为 1.3% (39/2893)、1.2% (21/1811) 和 0.2% (3/1888)分别为血小板增多症、真性红细胞增多症和原发性骨髓纤维化。脑静脉血栓形成发生在 ( n  = 20, 27%) 之前、( n  = 32, 44%) 或之后 ( n = 22) MPN 诊断。共有 72% 的患者出现头痛。在 10 名 (14%) 患者中发现中枢神经系统出血涉及横窦 (51%)、矢状窦 (43%) 和乙状窦 (35%)。总之，91% 的测试患者携带JAK2V617F。在 19 例 (31%) 病例中发现了潜在的血栓形成倾向，在 10 例 (14%) 中发现了血栓形成史。CVT 的治疗包括单独全身抗凝治疗 ( n  = 27) 或联合阿司匹林 ( n  = 24)、细胞减灭治疗 ( n  = 14) 或两者兼而有之 ( n  =  9 )）。在中位随访 5.1 年（范围 0.1-28.6）中，3 名（4%）患者出现 CVT 复发，而动脉和静脉血栓复发和大出血的比例分别为 11%、9% 和 14%。后续神经系统评估显示头痛 ( n  = 9)、视力丧失 ( n  = 1) 和认知障碍 ( n  = 1)。目前的研究明确了 MPN 相关的 CVT，并强调了它与JAK2 V617F、年轻年龄和女性的密切关系。区分 COVID 疫苗相关 CVT 与 MPN 相关 CVT 的临床特征包括，后者并发静脉血栓形成和脑出血的可能性较低；因此，MPN 相关的 CVT 并不致命。