Cerebral venous sinus thrombosis (CVST) is an uncommon venous thromboembolic event accounting for <1% of strokes resulting in brain parenchymal injuries. JAK2V617F mutation, the most frequent driving mutation of myeloproliferative neoplasms has been reported to be associated with worse clinical outcomes in patients with CVST. We investigated whether hematopoietic JAK2V617F expression predisposes to specific pathophysiological processes and/or worse prognosis after CVST. Using an in vivo mouse model of CVST, we analyzed clinical, biological and imaging outcomes in mice with hematopoietic-restricted Jak2V617F expression, compared to Jak2WT mice. In parallel, we studied a human cohort of JAK2V617F-positive or negative CVST. Early after CVST, mice with hematopoietic Jak2V617F expression had increased adhesion of platelets and neutrophils in cerebral veins located in the vicinity of CVST. On day 1, Jak2V617F mice had a worse outcome characterized by significantly more frequent and severe intracranial hemorrhages (ICH) and higher mortality rates. Peripheral neutrophil activation was enhanced, as indicated by higher circulating platelet-neutrophil aggregates, upregulated CD11b expression, and higher myeloperoxydase (MPO) plasma level. Concurrently, immunohistological and brain homogenates analysis showed higher neutrophil infiltration and increased blood-brain-barrier disruption. Similarly, JAK2V617F-positive CVST patients tended to present higher thrombotic burden and had significantly higher SII, a systemic thrombo-inflammatory marker, compared to JAK2V617F-negative patients. In mice with CVST, our study corroborates that Jak2V617F mutation leads to a specific pattern including increased thrombotic burden, ICH and mortality. The exacerbated thrombo-inflammatory response, observed both in mice and JAK2V617F-positive patients, could contribute to hemorrhagic complications.

Exacerbation of thrombo-inflammation by JAK2V617F mutation worsens the prognosis of cerebral venous sinus thrombosis.

The development of National clinical guidelines on diagnosis and treatment of Ph-negative myeloproliferative neoplasms provides the knowledge basis for decision making in diagnosis, therapy, prevention, and rehabilitation in compliance with evidence-based medicine principles in order to ensure appropriate medical care in a particular clinical setting. The clinical guidelines underly the choice of optimal methods of diagnosis, therapy, prevention, and rehabilitation as part of a patient’s medical care, the development of criteria for the assessment of medical care quality, medical care standards, medical care procedures, setting standards on equipment and management of diagnostic and therapeutic procedures, and provision of continuous medical education and advanced training for healthcare professionals. In 2013, a working group was formed to develop and formulate clinical guidelines on the treatment of myeloproliferative neoplasms. These guidelines were first published in 2014, afterwards they were revised and republished. The dynamic development of current hematology presupposes constant updating of knowledge and implementation of new diagnosis and treatment methods in clinical practice. In this context, clinical guidelines present a dynamic document to be continuously amended, expanded, and updated in accordance with scientific findings and new requirements of specialists who are directly involved in treatment activities. The present edition is an upgraded version of clinical guidelines with updated information on classification, diagnosis, prognosis, current methods of therapy, and their efficacy and tolerability assessment. The guidelines are intended for oncologists, hematologists, healthcare executives, and medical students. 

Национальные клинические рекомендации по диагностике и лечению Ph-негативных миелопролиферативных новообразований (истинной полицитемии, эссенциальной тромбоцитемии, первичного миелофиброза) (редакция 2024 г.)

Abstract Background and purpose We aimed to report the characteristics of cerebral venous thrombosis (CVT) in elderly people (aged ≥65 years). Methods This multicenter retrospective cohort included elderly patients hospitalized for a first CVT in nine Paris–Ile‐de‐France hospitals between 2011 and 2021. The estimated incidence was compared to CVT recorded by the French health insurance data system. Lariboisière Hospital's CVT registry allowed comparisons of our elderly cohort with individuals younger than 65 years. Results One hundred fourteen patients were included in this study (mean age = 74.2 years, range = 65–93, 61% female). The CVT annual incidence in Ile‐de‐France was 5.9–7.1 per million elderly individuals versus 8.5 per million nationwide. Headaches and focal deficits were the most common initial clinical features (50% and 51%, respectively), followed by seizures and confusion (40% and 27%). Treatment included anticoagulation (93%) and, rarely, endovascular procedure (2%) or craniectomy (1%). Compared with adult patients aged &lt;65 years (younger adults), elderly patients presented fewer headaches (50% vs. 96%, p &lt; 0.01) and intracranial hypertension (7% vs. 22%, p &lt; 0.01) but more seizures and focal deficits (40% vs. 27% and 51% vs. 38%, respectively, p &lt; 0.01). Underlying cancer, hemopathy, and locoregional infections were more frequent in elderly patients than among younger adults ( p &lt; 0.01). The prognosis of patients from our elderly cohort was poorer than that of younger adults; 8% died in the acute phase, and 73% had a favorable outcome at 1 year (vs. 1.7% and 87%, respectively, p &lt; 0.01). Conclusions CVT in elderly patients has a specific clinical presentation, epidemiology, and risk factors such as cancer or hemopathy, justifying specialized management. 

Cerebral venous thrombosis in elderly patients

Blood cell JAKtivation aggravates cerebral venous thrombosis

Importance One of 10 patients develop epilepsy in the late phase after cerebral venous thrombosis (CVT) diagnosis but predicting the individual risk is difficult. Objective To develop and externally validate a prognostic score to estimate the individual risk of post-CVT epilepsy. Design, Setting, and Participants This observational cohort study included both retrospective and prospective patients enrolled from 1994 through 2022. For development of the DIAS 3 score, data from the International CVT Consortium (n = 1128), a large international hospital-based multicenter CVT cohort, were used. For validation, data from 2 independent multicenter cohorts, the ACTION-CVT (n = 543) and the Israel CVT study (n = 556), were used. Of 2937 eligible, consecutively enrolled adult patients with radiologically verified CVT, 710 patients with a history of epilepsy prior to CVT, follow-up less than 8 days, and missing late seizure status were excluded. Exposure The prediction score (DIAS 3 ) was developed based on available literature and clinical plausibility and consisted of 6 readily available clinical variables collected during the acute phase: decompressive hemicraniectomy, intracerebral hemorrhage at presentation, age, seizure(s) in the acute phase (excluding status epilepticus), status epilepticus in the acute phase, and subdural hematoma at presentation. Main Outcome and Measure Time to a first late seizure, defined as occurring more than 7 days after diagnosis of CVT. Results Of 1128 patients included in the derivation cohort (median age, 41 [IQR, 30-53] years; 805 women [71%]), 128 (11%) developed post-CVT epilepsy during a median follow-up of 12 (IQR, 3-26) months. According to the DIAS 3 score, the predicted 1-year and 3-year risk of epilepsy in individual patients ranged from 7% to 68% and 10% to 83%, respectively. Internal and external validation showed adequate discrimination in the derivation cohort (1 year and 3 years: C statistic, 0.74; 95% CI, 0.70-0.79) and the 2 independent validation cohorts, (ACTION-CVT) 1 year: C statistic, 0.76; 95% CI, 0.67-0.84; 3 years: C statistic, 0.77; 95% CI, 0.66-0.84; and Israel CVT study 1 year: C statistic, 0.80; 95% CI, 0.75-0.86. Calibration plots indicated adequate agreement between predicted and observed risks. Conclusions and Relevance The DIAS 3 score (freely available online) is a simple tool that can help predict the risk of post-CVT epilepsy in individual patients. The model can improve opportunities for personalized medicine and may aid in decision-making regarding antiseizure medication, patient counseling, and facilitation of research on epileptogenesis in CVT. 

Development and Validation of a Clinical Score to Predict Epilepsy After Cerebral Venous Thrombosis

Characteristics of patients with cerebral sinus venous thrombosis and JAK2 V617F mutation.

Objectives. Cerebral sinus venous thrombosis (CSVT) is a rare stroke subtype and data regarding prognostic factors to predict outcomes are lacking. Thus, we aimed to identify predictors for outcome among CSVT patients. Materials and Methods. Prospective CSVT databases from four academic medical centers were retrospectively studied. Demographics, clinical presentations, risk factors, radiological, and outcome parameters were compared. Results. Out of 508 patients diagnosed with CSVT, 21 patients (4%) died, and 91 (18.6%) had unfavorable outcome (
 
 mRS
 ≥
 2
 
 ). Age (55.0 vs. 38.5, 
 
 p
 <
 0.001
 
 ), hypertension (26% vs. 6%, 
 
 p
 <
 0.001
 
 ), hyperlipidemia (23% vs. 6%, 
 
 p
 <
 0.001
 
 ), diabetes (17% vs. 4%, 
 
 p
 <
 0.001
 
 ), malignancy (35% vs. 11%, 
 
 p
 <
 0.001
 
 ), absence of headache (51% vs. 78%, 
 
 p
 <
 0.001
 
 ), focal neurological deficit (54% vs. 19%, 
 
 p
 <
 0.001
 
 ), and ICH (28% vs. 13%, 
 
 p
 <
 0.001
 
 ) were all associated with unfavorable outcome. After multivariate analysis malignancy (OR 4.2, 
 
 p
 =
 0.003
 
 ), the presence of focal neurological deficit (OR 5.2, 
 
 p
 <
 0.001
 
 ) and the presence of headache upon presentation (OR 0.334, 
 
 p
 =
 0.018
 
 ) remained significant predictors for favorable outcome. Conclusions. Among CSVT patients, malignancy, focal neurological deficits, and absence of headache at presentation were associated with unfavorable outcomes.

/pdf/prognosis-and-outcome-of-cerebral-sinus-venous-thrombosis-a-3etnesae.pdf

Prognosis and Outcome of Cerebral Sinus Venous Thrombosis—A Multicenter Cohort Study

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Characteristics of patients with cerebral sinus venous thrombosis and JAK2 V617F mutation.

Prognosis and Outcome of Cerebral Sinus Venous Thrombosis—A Multicenter Cohort Study