Survival and Disease Progression in Essential Thrombocythemia Are Significantly Influenced by Accurate Morphologic Diagnosis: An International Study
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- 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.
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- 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.
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- 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.
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- 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.
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- 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.
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- 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.
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- 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.
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- 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.
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- 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.
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- 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.
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- 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.
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- 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.
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- 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.
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- 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.
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- 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.
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- 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.
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- 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.
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- 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.
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- 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.
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- 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.
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- 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.
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- 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.
説明
<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>
収録刊行物
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- Journal of Clinical Oncology
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Journal of Clinical Oncology 29 (23), 3179-3184, 2011-08-10
American Society of Clinical Oncology (ASCO)