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• W2120336245 abstract "The diagnosis of the myeloproliferative neoplasms (MPN)‒ essential thrombocythaemia (ET) and primary myelofibrosis (PMF) ‒ in patients lacking a molecular marker is challenging. Recently, mutations in exon 9 of the calreticulin gene (CALR) have been described in around one-third of ET and MF patients (Klampfl et al, 2013a; Nangalia et al, 2013). Notably, these mutations are almost always seen in JAK2 V617F-negative and MPL-non-mutated patients and account for the majority of these cases. Such is the prevalence of these mutations we suggest that they are added to the British Committee for Standards in Haematology criteria for the diagnosis of ET and also PMF (Harrison et al, 2010; Reilly et al, 2012) (evidence grade 1A). The modified diagnostic criteria and algorithms are shown in Table 1 and Fig 1. Some initial reports suggest that the presence of a CALR mutation is associated with greater longevity and lower risk of thrombosis in ET patients (Klampfl et al, 2013b) (Rotunno et al, 2014). However the utility of incorporating CALR mutation status into current risk stratification is not yet tested and therefore is not recommended. Furthermore, the guideline writing group also considers the utility of the proposed International Prognostic Score for ET (IPS ET) to be limited and does not recommend its incorporation into standard practice (Barbui et al, 2012; Passamonti et al, 2012). The reasons for this decision are that the score was tested upon retrospective selected groups of patients who were already receiving treatment. The application of IPS ET results in re-allocation of large proportions of patients from high risk into low and intermediate and vice versa. The management of intermediate risk ET is uncertain and has only been tested in the PT-1 intermediate risk study, which has not yet been reported and uses a different definition of intermediate risk. The authors have no conflicts of interest to disclose. All authors have contributed equally to this document." @default.
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• W2120336245 date "2014-06-17" @default.
• W2120336245 modified "2023-10-04" @default.
• W2120336245 title "Modification of British Committee for Standards in Haematology diagnostic criteria for essential thrombocythaemia" @default.
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• W2120336245 doi "https://doi.org/10.1111/bjh.12986" @default.
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