Decrease in JAK2 V617F allele burden is not a prerequisite to clinical response in patients with polycythemia vera |
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Authors: | Kuriakose Emil Vandris Katherine Wang Y Lynn Chow William Jones Amy V Christos Paul Cross Nicholas C P Silver Richard T |
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Institution: | Weill Cornell Medical College, Department of Medicine, Division of Hematology and Medical Oncology, New York, NY 10021, USA. |
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Abstract: | BackgroundAlthough reduction in the JAK2V617F allele burden (%V617F) has been suggested as a criterion for defining disease response to cytoreductive therapy in polycythemia vera, its value as a response monitor is unclear. The purpose of this study is to determine whether a reduction in %V617F in polycythemia vera is a prerequisite to achieving hematologic remission in response to cytoreductive therapy.Design and MethodsWe compared the clinical and hematologic responses to change in %V617F (molecular response) in 73 patients with polycythemia vera treated with either interferon (rIFNα-2b: 28, Peg-rIFNα-2a: 18) or non-interferon drugs (n=27), which included hydroxyurea (n=8), imatinib (n=12), dasatinib (n=5), busulfan (n=1), and radioactive phosphorus (n=1). Hematologic response evaluation employed Polycythemia Vera Study Group criteria, and molecular response evaluation, European Leukemia Net criteria.ResultsOf the 46 treated with interferon, 41 (89.1%) had a hematologic response, whereas only 7 (15.2%) had a partial molecular response. Of the 27 who received non-interferon treatments, 16 (59.3%) had a hematologic response, but only 2 (7.4%) had a molecular response. Median duration of follow up was 2.8 years. Statistical agreement between hematologic response and molecular response was poor in all treatment groups.ConclusionsGenerally, hematologic response was not accompanied by molecular response. Therefore, a quantitative change in %V617F is not required for clinical response in patients with polycythemia vera. |
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Keywords: | JAK2V617F molecular response polycythemia vera reduced allele burden |
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