A prospective study of 338 patients with polycythemia vera: the impact of JAK2 (V617F) allele burden and leukocytosis on fibrotic or leukemic disease transformation and vascular complications

Leukemia
Francesco PassamontiMario Cazzola

Abstract

We studied the relationship between JAK2 (V617F) mutant allele burden and clinical phenotype, disease progression and survival in patients with polycythemia vera (PV). The percentage of granulocyte mutant alleles was evaluated using a quantitative real-time polymerase chain reaction-based allelic discrimination assay. Of the 338 patients enrolled in this prospective study, 320 (94.7%) carried the JAK2 (V617F) mutation. Direct relationships were found between mutant allele burden and hemoglobin concentration (P=0.001), white blood cell count (P=0.001), spleen size (P=0.001) and age-adjusted bone marrow cellularity (P=0.002), while an inverse relationship was found with platelet count (P<0.001). During the study period, eight patients progressed to post-PV myelofibrosis (MF) (all carrying >50% mutant alleles), while 10 patients developed acute myeloid leukemia (AML). The mutant allele burden was significantly related to the risk of developing myelofibrosis (P=0.029) and retained its significant effect also in multivariable analysis (P=0.03). By contrast, the risk of developing AML as well as that of thrombosis was not significantly related to mutant allele burden. Leukocytosis did not affect thrombosis, MF, leukemia or survival. ...Continue Reading

References

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Citations

Jan 27, 2012·Current Hematologic Malignancy Reports·Giovanni BarosiVittorio Rosti
Jun 22, 2013·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Leonardo BoiocchiAttilio Orazi
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Mar 29, 2014·International Journal of Hematology·Yoko EdahiroNorio Komatsu
Sep 6, 2014·Best Practice & Research. Clinical Haematology·Francesco PassamontiMargherita Maffioli
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Jan 31, 2016·Current Opinion in Hematology·Francesco PassamontiMargherita Maffioli
Dec 10, 2015·Experimental Hematology·Linghua WangJosef T Prchal

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