Incidence and risk factors for myelofibrotic transformation among 272 Chinese patients with JAK2-mutated polycythemia vera

Abstract

This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process which may lead to differences between this version and the Version of Record. Please cite this article as an 'Accepted Article', doi: 10.1002/ajh.24182This article is protected by copyright. All rights reserved.

Thrombosis among 1537 patients with JAK2V617F‐mutated myeloproliferative neoplasms: Risk factors and development of a predictive model

“…MPNs mainly include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) . Thrombosis, leukemic transformation, and myelofibrotic transformation are critical complications of MPN and are highly common in patients with MPN . Fatalities due to thrombosis account for 35%~70% of the total mortality rate of MPN, making it the most important risk factor affecting event‐free survival and overall survival (OS) of MPN patients …”

“…1 Thrombosis, leukemic transformation, and myelofibrotic transformation are critical complications of MPN and are highly common in patients with MPN. [2][3][4][5][6] Fatalities due to thrombosis account for 35%~70% of the total mortality rate of MPN, making it the most important risk factor affecting eventfree survival and overall survival (OS) of MPN patients. 7 Studies in the last decade have shown that JAK2/CALR/MPL gene mutations are the driver mutations in MPN.…”

JAK2 V617F as a Marker for Long-Term Disease Progression and Mortality in Polycythemia Vera and its Role in Economic Modeling

“…When we combined risk levels to establish a range of possible OS levels, our model provided an OS range of 14.2 to 17.3 LY depending on age and baseline JAK2. Only the study by Bai et al 19 had an OS (17.5 LY) that slightly fell outside of the modeled OS range.…”

“…We developed an economic model for PV that combines data from multiple sources such as clinical trials and long-term observational cohort studies. 12,15,[17][18][19][20][21][22][23][24][25][26][27][28][29][30] The objective of this study was to validate the model that uses JAK2 burden as a surrogate endpoint to predict DP and OS based on progression-specific mortality.…”

“…The results of the validation indicated a good correspondence between the model and the published observational studies available for comparisons. The mean OS in the published observational studies 19,31,32 that was included in the validation of the model was 14.9 to 17.5 years over a 20-year time horizon. When we adjusted our model's baseline age and baseline JAK2 burden to match the studies' variables, our model predicted a mean OS that ranged between 15.0 to 16.5 years.…”