Cerebral venous thrombosis and myeloproliferative neoplasms: a three-center study of 74 consecutive cases.

Gangat, Naseema; Guglielmelli, Paola; Betti, Silvia; Farrukh, Faiqa; Carobbio, Alessandra; Barbui, Tiziano; Vannucchi, Alessandro M; DeStefano, Valerio; Tefferi, Ayalew

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 (MPN), seen at the Mayo Clinic, Catholic University of Rome, and University of Florence, between 1991 and 2021. Disease-specific frequencies were 1.3% (39/2,893), 1.2% (21/1,811) and 0.2% (3/1,888) for essential thrombocythemia, polycythemia vera and primary myelofibrosis, respectively. CVT occurred either prior to (n=20, 27%), at (n=32, 44%) or after (n=22) MPN diagnosis. 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. 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 3 (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 on MPN-associated CVT and highlights its close association with JAK2V617F, younger age, and female gender. Clinical features that distinguish COVID vaccine-related from MPN-CVT include lower likelihood of concurrent venous thromboses and intracerebral hemorrhage with the latter; as a result, MPN- CVT was not fatal. This article is protected by copyright. All rights reserved.