Cerebral venous thrombosis and myeloproliferative neoplasms: A three‐center study of 74 consecutive cases. Issue 12 (10th September 2021)

• Record Type: Journal Article
• Authors: Gangat, Naseema; Guglielmelli, Paola; Betti, Silvia; Farrukh, Faiqa; Carobbio, Alessandra; Barbui, Tiziano; Vannucchi, Alessandro M.; De Stefano, Valerio; Tefferi, Ayalew
• Is Part Of: American journal of hematology. Volume 96:Issue 12(2021)
• Page Start: 1580
• Page End: 1586
• Publication Date: 2021-09-10
• Subjects: Hematology -- Periodicals; 616.15
• DOI: 10.1002/ajh.26336
• Languages: English
• ISSNs: 0361-8609

Abstract

Abstract: 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 JAK2 V617F, 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.