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      Long-term survival and blast transformation in molecularly annotated essential thrombocythemia, polycythemia vera, and myelofibrosis.

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          Abstract

          Janus kinase 2 (JAK2) mutations define polycythemia vera (PV). Calreticulin (CALR) and myeloproliferative leukemia virus oncogene (MPL) mutations are specific to JAK2-unmutated essential thrombocythemia (ET) and primary myelofibrosis (PMF). We examined the effect of these mutations on long-term disease outcome. One thousand five hundred eighty-one patients from the Mayo Clinic (n = 826) and Italy (n = 755) were studied. Fifty-eight percent of Mayo patients were followed until death; median survivals were 19.8 years in ET (n = 292), 13.5 PV (n = 267; hazard ratio [HR], 1.8; 95% confidence interval [CI], 1.4-2.2), and 5.9 PMF (n = 267; HR, 4.5; 95% CI, 3.5-5.7). The survival advantage of ET over PV was not affected by JAK2/CALR/MPL mutational status. Survival in ET was inferior to the age- and sex-matched US population (P < .001). In PMF (n = 428), but not in ET (n = 576), survival and blast transformation (BT) were significantly affected by mutational status; outcome was best in CALR-mutated and worst in triple-negative patients: median survival, 16 vs 2.3 years (HR, 5.1; 95% CI, 3.2-8.0) and BT, 6.5% vs 25% (HR, 7.6; 95% CI, 2.8-20.2), respectively. We conclude that life expectancy in morphologically defined ET is significantly reduced but remains superior to that of PV, regardless of mutational status. In PMF, JAK2/CALR/MPL mutational status is prognostically informative.

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          Author and article information

          Journal
          Blood
          Blood
          1528-0020
          0006-4971
          Oct 16 2014
          : 124
          : 16
          Affiliations
          [1 ] Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN;
          [2 ] Laboratorio Congiunto MMPC, Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy;
          [3 ] Department of Statistics and Bioinformatics.
          [4 ] Division of Cytogenetics, Department of Laboratory Medicine, and.
          [5 ] Division of Hematopathology, Department of Laboratory Medicine, Mayo Clinic, Rochester, MN;
          [6 ] Division of Hematology and Research Foundation, Papa Giovanni XXIII Hospital, Bergamo, Italy; and.
          [7 ] Institute of Pathology, University of Cologne, Cologne, Germany.
          Article
          blood-2014-05-579136
          10.1182/blood-2014-05-579136
          4199952
          25037629
          2f0d3ca0-9abc-4af9-80c1-b380a880c52d
          © 2014 by The American Society of Hematology.
          History

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