JAK2 V617F Genotype Is a Strong Determinant of Blast Transformation in Primary MyelofibrosisReportar como inadecuado

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The influence of JAK2 V617F mutation on blast transformation BT and overall survival OS in primary myelofibrosis PMF is controversial. In a large cohort of patients we applied competing risks analysis for studying the influence of JAK2V617F mutation on BT in PMF.

Patients and Methods

In 462 PMF–fibrotic type patients bone marrow BM fibrosis grade >0 we computed the incidence of BT and death in the framework of Cox regression analysis and of Fine and Gray competing risks analysis for BT.


At the Cox regression analysis, having either a wild-type wt or a homozygous JAK2V617F genotype were factors for BT HR, 1.98 and 2.04, respectively, with respect to the heterozygous genotype, but not for OS. At the competing risks regression analysis, the risk for BT in wt and homozygous V617F patients increased with respect to Cox analysis, giving a sHR of 2.17 and 2.12, respectively. Correcting the results for the variables that could have influence on BT, JAK2V617F wt and homozygous genotypes remained independently associated with BT. In a validation cohort of 133 independent cases with PMF-prefibrotic type BM fibrosis grade  = 0, the BT predictive model including JAK2V617F genotype and older age retained high discriminant capacity C statistics, 0.70; 95% CI, 0.47 to 0.92.


The accumulation of mutated alleles in the JAK2V617F clone or the selective acquisition of a proliferative advantage in the wt clone are two relevant routes to BT in PMF. The influence of these results on treatment decisions with anti-JAK2 agents should be tested.

Autor: Giovanni Barosi , Valentina Poletto, Margherita Massa, Rita Campanelli, Laura Villani, Elisa Bonetti, Gianluca Viarengo, Paolo Ca

Fuente: http://plos.srce.hr/


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