The most characteristic of these disturbances is erythromelalgia,

The most characteristic of these disturbances is erythromelalgia, consisting of congestion, redness and burning pain involving the extremities. On the basis of several prospective and retrospective cohort studies in PV and ET, age older than 60 years and previous thrombosis have been identified as major predictors of vascular complications.[19] and [20] Moreover, there is evidence that leukocytosis and JAK2 mutation

may be included Selleck Afatinib in the prognostic stratification provided new studies will confirm their predictive role. Experts of the European LeukemiaNet group (ELN) agree that a clear association between platelet counts and major vascular events is lacking and that extreme thrombocytosis (i.e., > 1500 × 109/L) can be associated with acquired von Willebrand disease and bleeding tendency.[12] and [21] The relation between hematocrit levels up to 50% and thrombosis is uncertain.22 check details By incorporating this body of knowledge in a clinically oriented scheme (Table 1), patients with either PV or ET can be stratified in a “high-risk” or “low-risk” category according to their age and previous history of thrombosis; an “intermediate-risk” category,

that would include younger patients with coexisting generic cardiovascular risk factors in the absence of previous thrombosis, is also defined, but formal proof of its relevance to stratify patients is still lacking. It should be underlined that these concepts are based on relative risk estimates such as odds ratio, risk ratio, or hazard ratio so that no Buspirone HCl direct meaning or relevance to prognostication of thrombosis in individual patient can be drawn by these tools. In fact, given the variability among

ET and PV patients in the clinical and hematologic presentation and treatment of the disease, a single predictor or variable even though generated by a multivariable approach rarely gives an adequate estimate of thrombotic prediction in individual patient or groups. In contrast, a more reliable and consistent prediction of thrombotic risk may be provided by combining multiple variables in prognostic models whose performance needs to be confirmed in other cohorts of patients. In primary myelofibrosis (PMF), such studies are available,23 but we need this information in PV and ET as well. Thrombosis is a multifactorial process and its pathogenesis results from an interplay of various factors other than the myeloproliferative disease. The identification and appropriate management of cardiovascular risk factors and the promotion of a healthy lifestyle in MPN, as in the general population, should be considered a cornerstone of vascular prevention. Particular attention has to be given to smoking habit which has an important effect on vascular risk and which was found to be surprisingly common among PV patients recruited in the ECLAP observational study.

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