Cerebral vein thrombosis in patients with <scp>P</scp>hiladelphia‐negative myeloproliferative neoplasms An <scp>E</scp>uropean <scp>L</scp>eukemia <scp>N</scp>et study

Martinelli, Ida; Stefano, Valerio De; Carobbio, Alessandra; Randi, Maria Luigia; Sommer, Claudia; Rambaldi, Alessandro; Finazzi, Maria Chiara; Cervantes, Francisco; Arellano‐Rodrigo, Eduardo; Rupoli, Serena; Canafoglia, Lucia; Tieghi, Alessia; Facchini, Luca; Betti, Silvia; Vannucchi, Alessandro M.; Pieri, Lisa; Cacciola, Rossella; Cacciola, Emma; Cortelezzi, Agostino; Iurlo, Alessandra; Pogliani, Enrico Maria; Elli, Elena Maria; Spadea, Antonio; Barbui, Tiziano

doi:10.1002/ajh.23809

Cited by 44 publications

(61 citation statements)

References 21 publications

(21 reference statements)

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“…In a series of 48 patients with MPN and CVT, diagnosis of MPN was concomitant to that of CVT in 46% of the patients, and 81% of JAK2 V617F positivity was observed [28]. …”

Section: Discussionmentioning

confidence: 99%

“…About 30% of vascular events in Ph-negative MPN patients are VTE [27], which generally present as lower limb deep vein thrombosis or pulmonary artery embolism. However, in Ph-negative MPN, VTE characteristically occurs in uncommon locations such as splanchnic veins, including hepatic veins (presenting Budd-Chiari syndrome), portal and mesenteric veins, or, less frequently, cerebral veins or sinuses [15,28]. In patients with MPN, the prevalence of CVT is 1% or less [23,29,30], and among patients with CVT, MPN is concomitantly diagnosed in 3-7% of cases [23,31,32].…”

Section: Discussionmentioning

confidence: 99%

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Should We Screen for Janus Kinase 2 V617F Mutation in Cerebral Venous Thrombosis?

Lamy¹,

Palazzo

Agius³

et al. 2017

Cerebrovasc Dis

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Background: The presence of Janus Kinase 2 (JAK2) V617F mutation represents a major diagnostic criterion for detecting myeloproliferative neoplasms (MPN) and even in the absence of overt MPN, JAK2 V617F mutation is associated with splanchnic vein thrombosis. However, the actual prevalence and diagnostic value of the JAK2 V617F mutation in patients with cerebral venous thrombosis (CVT) are not known. The aims of this study were to assess the prevalence of JAK2 V617F mutation in a large group of consecutive CVT patients, to detect clinical, biological, and radiological features associated with the mutation, and to determine the long-term venous thrombosis recurrence rate in CVT patients with JAK2 mutation but without overt MPN in order to recommend the best preventive treatment. Methods: This was a prospective study conducted on consecutive patients with a first-ever radiologically confirmed CVT. JAK2 V617F mutation analysis was assessed in all the study subjects. JAK2 V617F-positive patients were followed up to detect new venous thrombotic events. Results: Of the 125 included subjects, 7 were found to have JAK2 V617F mutation (5.6%; 95% CI 2.3-11.2). Older age (p = 0.039) and higher platelet count (p = 0.004) were independently associated with JAK2 V617F positivity in patients without overt MPN. During a mean follow-up period of 59 (SD 46) months, 2 JAK2 V617F-positive patients presented with 4 new venous thromboembolic events. Conclusions: Screening for the JAK2 V617F mutation in CVT patients seems to be useful even in the absence of overt MPN and/or in the presence of other risk factors for CVT because of its relatively high prevalence and the risk of thrombosis recurrence.

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“…In a series of 48 patients with MPN and CVT, diagnosis of MPN was concomitant to that of CVT in 46% of the patients, and 81% of JAK2 V617F positivity was observed [28]. …”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Should We Screen for Janus Kinase 2 V617F Mutation in Cerebral Venous Thrombosis?

Lamy¹,

Palazzo

Agius³

et al. 2017

Cerebrovasc Dis

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“…82 Cerebral vein thrombosis is a rare, life-threatening event that leads to a concurrent diagnosis of a MPN in 2% to 7% of cases; the estimated prevalence among all MPN is 1% to 2%. [83][84][85] A slightly higher frequency of cerebral vein thrombosis has been reported in PV than ET. 84 Resistance/refractoriness to first-line treatment Over time, 10% to 15% of patients on hydroxyurea develop hematologic and extrahematologic (painful leg ulcers, skin cancers, or extensive dermatitis, and fever) toxicities 86 and have to stop the drug or use a suboptimal dose (Table 6 87,88 ).…”

Section: Special Situations Of Interest Thrombosis In Unusual Sitesmentioning

confidence: 99%

How I treat polycythemia vera

Vannucchi

2014

Blood

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Polycythemia vera (PV) is a chronic myeloproliferative neoplasm associated with JAK2 mutations (V617F or exon 12) in almost all cases. The World Health Organization has defined the criteria for diagnosis, but it is still unclear which parameter (hemoglobin or hematocrit) is the most reliable for demonstrating increased red cell volume and for monitoring response to therapy; also, the role of bone marrow biopsy is being revisited. PV is associated with reduced survival because of cardiovascular complications and progression to post-PV myelofibrosis or leukemia. Criteria for risk-adapted treatment rely on the likelihood of thrombosis. Controlled trials have demonstrated that incidence of cardiovascular events is reduced by sustained control of hematocrit with phlebotomies (low-risk patients) and/or cytotoxic agents (high-risk patients) and antiplatelet therapy with aspirin. Hydroxyurea and interferon may be used as first-line treatments, whereas busulfan is reserved for patients that are refractory or resistant to first-line agents. However, there is no evidence that therapy improves survival, and the significance of reduction of JAK2 mutated allele burden produced by interferon is unknown. PV is also associated with a plethora of symptoms that are poorly controlled by conventional therapy. This article summarizes my approach to the management of PV in daily clinical practice.

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“…Intriguingly, BCS, which is caused by thrombotic obstruction of the hepatic veins and/ or the suprahepatic inferior vena cava, has been attributed to an MPN in 41 % of cases, while 30 % of patients with portal vein thrombosis have a diagnosis of MPN (11). The prevalence of CVST in MPN patients is 1 %, and in nearly half of the patients, the diagnoses are made concomitantly (12).…”

Section: Discussionmentioning

confidence: 99%

“…However, MPN patients were not included in these studies (28). Venous thromboses in atypical locations such as CVST are associated with a high risk of recurrence and patients should therefore be evaluated for long-term anticoagulation with VKA (12,28). Intriguingly, a most recent publication supports the use of longterm anticoagulation for secondary prevention of MPN-associated VTE, regardless of the site of the initial thrombotic event (29).…”

Section: Polycythemia Veramentioning

confidence: 99%