This study investigates disease characteristics and clinical outcome in young patients (<40 years) with WHO-defined essential thrombocythemia (ET) in comparison to early/prefibrotic primary myelofibrosis (PMF) with presenting thrombocythemia. We recruited 213 young patients (median age 33.6 years) including 178 patients (84%) consistent with WHO-defined ET and 35 patients (16%) showing early PMF. Median follow-up was 7.5 years. A trend for more overall thrombotic, particularly arterial, complications was seen in early PMF compared to ET. Progression to overt myelofibrosis was 3% in ET and 9% in early PMF, but no transformation into acute leukemia was observed. By combining all adverse events (thrombosis, bleeding, myelofibrosis) the rate was significantly different (1.29 vs. 3.43% patients/year, p=0.01) in WHO-ET and early PMF, respectively. In multivariate analysis early PMF and JAK2V617F mutation emerged as independent factors in predicting cumulative adverse events.

Disease characteristics and clinical outcome in young adults with essential thrombocythemia versus early /prefibrotic primary myelofibrosis.

RUMI, ELISA;
2012-01-01

Abstract

This study investigates disease characteristics and clinical outcome in young patients (<40 years) with WHO-defined essential thrombocythemia (ET) in comparison to early/prefibrotic primary myelofibrosis (PMF) with presenting thrombocythemia. We recruited 213 young patients (median age 33.6 years) including 178 patients (84%) consistent with WHO-defined ET and 35 patients (16%) showing early PMF. Median follow-up was 7.5 years. A trend for more overall thrombotic, particularly arterial, complications was seen in early PMF compared to ET. Progression to overt myelofibrosis was 3% in ET and 9% in early PMF, but no transformation into acute leukemia was observed. By combining all adverse events (thrombosis, bleeding, myelofibrosis) the rate was significantly different (1.29 vs. 3.43% patients/year, p=0.01) in WHO-ET and early PMF, respectively. In multivariate analysis early PMF and JAK2V617F mutation emerged as independent factors in predicting cumulative adverse events.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11571/449563
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