Survival and Disease Progression in Essential Thrombocythemia Are Significantly Influenced by Accurate Morphologic Diagnosis: An International Study

DOI Web Site 13 Citations
  • Tiziano Barbui
    From the Ospedali Riuniti di Bergamo, Bergamo; University of Pavia, Istituto Di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia; S. Bortolo Hospital, Vicenza; University of Padua, Padua; University of Florence, Florence, Italy; University of Cologne, Cologne, Germany; Medical University of Vienna, Vienna, Austria; and Mayo Clinic, Rochester, MN.
  • Juergen Thiele
    From the Ospedali Riuniti di Bergamo, Bergamo; University of Pavia, Istituto Di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia; S. Bortolo Hospital, Vicenza; University of Padua, Padua; University of Florence, Florence, Italy; University of Cologne, Cologne, Germany; Medical University of Vienna, Vienna, Austria; and Mayo Clinic, Rochester, MN.
  • Francesco Passamonti
    From the Ospedali Riuniti di Bergamo, Bergamo; University of Pavia, Istituto Di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia; S. Bortolo Hospital, Vicenza; University of Padua, Padua; University of Florence, Florence, Italy; University of Cologne, Cologne, Germany; Medical University of Vienna, Vienna, Austria; and Mayo Clinic, Rochester, MN.
  • Elisa Rumi
    From the Ospedali Riuniti di Bergamo, Bergamo; University of Pavia, Istituto Di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia; S. Bortolo Hospital, Vicenza; University of Padua, Padua; University of Florence, Florence, Italy; University of Cologne, Cologne, Germany; Medical University of Vienna, Vienna, Austria; and Mayo Clinic, Rochester, MN.
  • Emanuela Boveri
    From the Ospedali Riuniti di Bergamo, Bergamo; University of Pavia, Istituto Di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia; S. Bortolo Hospital, Vicenza; University of Padua, Padua; University of Florence, Florence, Italy; University of Cologne, Cologne, Germany; Medical University of Vienna, Vienna, Austria; and Mayo Clinic, Rochester, MN.
  • Marco Ruggeri
    From the Ospedali Riuniti di Bergamo, Bergamo; University of Pavia, Istituto Di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia; S. Bortolo Hospital, Vicenza; University of Padua, Padua; University of Florence, Florence, Italy; University of Cologne, Cologne, Germany; Medical University of Vienna, Vienna, Austria; and Mayo Clinic, Rochester, MN.
  • Francesco Rodeghiero
    From the Ospedali Riuniti di Bergamo, Bergamo; University of Pavia, Istituto Di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia; S. Bortolo Hospital, Vicenza; University of Padua, Padua; University of Florence, Florence, Italy; University of Cologne, Cologne, Germany; Medical University of Vienna, Vienna, Austria; and Mayo Clinic, Rochester, MN.
  • Emanuele S.G. d'Amore
    From the Ospedali Riuniti di Bergamo, Bergamo; University of Pavia, Istituto Di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia; S. Bortolo Hospital, Vicenza; University of Padua, Padua; University of Florence, Florence, Italy; University of Cologne, Cologne, Germany; Medical University of Vienna, Vienna, Austria; and Mayo Clinic, Rochester, MN.
  • Maria Luigia Randi
    From the Ospedali Riuniti di Bergamo, Bergamo; University of Pavia, Istituto Di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia; S. Bortolo Hospital, Vicenza; University of Padua, Padua; University of Florence, Florence, Italy; University of Cologne, Cologne, Germany; Medical University of Vienna, Vienna, Austria; and Mayo Clinic, Rochester, MN.
  • Irene Bertozzi
    From the Ospedali Riuniti di Bergamo, Bergamo; University of Pavia, Istituto Di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia; S. Bortolo Hospital, Vicenza; University of Padua, Padua; University of Florence, Florence, Italy; University of Cologne, Cologne, Germany; Medical University of Vienna, Vienna, Austria; and Mayo Clinic, Rochester, MN.
  • Filippo Marino
    From the Ospedali Riuniti di Bergamo, Bergamo; University of Pavia, Istituto Di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia; S. Bortolo Hospital, Vicenza; University of Padua, Padua; University of Florence, Florence, Italy; University of Cologne, Cologne, Germany; Medical University of Vienna, Vienna, Austria; and Mayo Clinic, Rochester, MN.
  • Alessandro M. Vannucchi
    From the Ospedali Riuniti di Bergamo, Bergamo; University of Pavia, Istituto Di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia; S. Bortolo Hospital, Vicenza; University of Padua, Padua; University of Florence, Florence, Italy; University of Cologne, Cologne, Germany; Medical University of Vienna, Vienna, Austria; and Mayo Clinic, Rochester, MN.
  • Elisabetta Antonioli
    From the Ospedali Riuniti di Bergamo, Bergamo; University of Pavia, Istituto Di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia; S. Bortolo Hospital, Vicenza; University of Padua, Padua; University of Florence, Florence, Italy; University of Cologne, Cologne, Germany; Medical University of Vienna, Vienna, Austria; and Mayo Clinic, Rochester, MN.
  • Valentina Carrai
    From the Ospedali Riuniti di Bergamo, Bergamo; University of Pavia, Istituto Di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia; S. Bortolo Hospital, Vicenza; University of Padua, Padua; University of Florence, Florence, Italy; University of Cologne, Cologne, Germany; Medical University of Vienna, Vienna, Austria; and Mayo Clinic, Rochester, MN.
  • Heinz Gisslinger
    From the Ospedali Riuniti di Bergamo, Bergamo; University of Pavia, Istituto Di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia; S. Bortolo Hospital, Vicenza; University of Padua, Padua; University of Florence, Florence, Italy; University of Cologne, Cologne, Germany; Medical University of Vienna, Vienna, Austria; and Mayo Clinic, Rochester, MN.
  • Veronika Buxhofer-Ausch
    From the Ospedali Riuniti di Bergamo, Bergamo; University of Pavia, Istituto Di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia; S. Bortolo Hospital, Vicenza; University of Padua, Padua; University of Florence, Florence, Italy; University of Cologne, Cologne, Germany; Medical University of Vienna, Vienna, Austria; and Mayo Clinic, Rochester, MN.
  • Leonhard Müllauer
    From the Ospedali Riuniti di Bergamo, Bergamo; University of Pavia, Istituto Di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia; S. Bortolo Hospital, Vicenza; University of Padua, Padua; University of Florence, Florence, Italy; University of Cologne, Cologne, Germany; Medical University of Vienna, Vienna, Austria; and Mayo Clinic, Rochester, MN.
  • Alessandra Carobbio
    From the Ospedali Riuniti di Bergamo, Bergamo; University of Pavia, Istituto Di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia; S. Bortolo Hospital, Vicenza; University of Padua, Padua; University of Florence, Florence, Italy; University of Cologne, Cologne, Germany; Medical University of Vienna, Vienna, Austria; and Mayo Clinic, Rochester, MN.
  • Andrea Gianatti
    From the Ospedali Riuniti di Bergamo, Bergamo; University of Pavia, Istituto Di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia; S. Bortolo Hospital, Vicenza; University of Padua, Padua; University of Florence, Florence, Italy; University of Cologne, Cologne, Germany; Medical University of Vienna, Vienna, Austria; and Mayo Clinic, Rochester, MN.
  • Naseema Gangat
    From the Ospedali Riuniti di Bergamo, Bergamo; University of Pavia, Istituto Di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia; S. Bortolo Hospital, Vicenza; University of Padua, Padua; University of Florence, Florence, Italy; University of Cologne, Cologne, Germany; Medical University of Vienna, Vienna, Austria; and Mayo Clinic, Rochester, MN.
  • Curtis A. Hanson
    From the Ospedali Riuniti di Bergamo, Bergamo; University of Pavia, Istituto Di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia; S. Bortolo Hospital, Vicenza; University of Padua, Padua; University of Florence, Florence, Italy; University of Cologne, Cologne, Germany; Medical University of Vienna, Vienna, Austria; and Mayo Clinic, Rochester, MN.
  • Ayalew Tefferi
    From the Ospedali Riuniti di Bergamo, Bergamo; University of Pavia, Istituto Di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia; S. Bortolo Hospital, Vicenza; University of Padua, Padua; University of Florence, Florence, Italy; University of Cologne, Cologne, Germany; Medical University of Vienna, Vienna, Austria; and Mayo Clinic, Rochester, MN.

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<jats:sec><jats:title>Purpose</jats:title><jats:p>The WHO diagnostic criteria underscore the role of bone marrow (BM) morphology in distinguishing essential thrombocythemia (ET) from early/prefibrotic primary myelofibrosis (PMF). This study examined the clinical relevance of such a distinction.</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>Representatives from seven international centers of excellence for myeloproliferative neoplasms convened to create a clinicopathologic database of patients previously diagnosed as having ET (N = 1,104). Study eligibility criteria included availability of treatment-naive BM specimens obtained within 1 year of diagnosis. All bone marrows subsequently underwent a central re-review.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>Diagnosis was confirmed as ET in 891 patients (81%) and was revised to early/prefibrotic PMF in 180 (16%); 33 patients were not evaluable. In early/prefibrotic PMF compared with ET, the 10-year survival rates (76% and 89%, respectively) and 15-year survival rates (59% and 80%, respectively), leukemic transformation rates at 10 years (5.8% and 0.7%, respectively) and 15 years (11.7% and 2.1%, respectively), and rates of progression to overt myelofibrosis at 10 years (12.3% and 0.8%, respectively) and 15 years (16.9% and 9.3%) were significantly worse. The respective death, leukemia, and overt myelofibrosis incidence rates per 100 patient-years for early/prefibrotic PMF compared with ET were 2.7% and 1.3% (relative risk [RR], 2.1; P < .001), 0.6% and 0.1% (RR, 5.2; P = .001), and 1% and 0.5% (RR, 2.0; P = .04). Multivariable analysis confirmed these findings and also identified age older than 60 years (hazard ratio [HR], 6.7), leukocyte count greater than 11 × 10<jats:sup>9</jats:sup>/L (HR, 2.01), anemia (HR, 2.95), and thrombosis history (HR, 2.81) as additional risk factors for survival. Thrombosis and JAK2V617F incidence rates were similar between the two groups. Survival in ET was similar to the sex- and age-standardized European population.</jats:p></jats:sec><jats:sec><jats:title>Conclusion</jats:title><jats:p>This study validates the clinical relevance of strict adherence to WHO criteria in the diagnosis of ET and provides important information on survival, disease complication rates, and prognostic factors in strictly WHO-defined ET and early/prefibrotic PMF.</jats:p></jats:sec>

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