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

被引：63

作者：

Barbui, Tiziano ^{[1
]}

Thiele, Juergen ^{[2
]}

Carobbio, Alessandra ^{[1
]}

Passamonti, Francesco ^{[3
]}

Rumi, Elisa ^{[4
]}

Randi, Maria Luigia ^{[5
]}

Bertozzi, Irene ^{[5
]}

Vannucchi, Alessandro M. ^{[6
]}

Gisslinger, Heinz

Gisslinger, Bettina ^{[7
]}

Finazzi, Guido ^{[1
]}

Ruggeri, Marco ^{[8
]}

Rodeghiero, Francesco ^{[8
]}

Rambaldi, Alessandro ^{[1
]}

Gangat, Naseema ^{[9
]}

Tefferi, Ayalew ^{[9
]}

机构：

[1] Osped Riuniti Bergamo, I-24100 Bergamo, Italy

[2] Univ Cologne, D-50931 Cologne, Germany

[3] Osped Varese, Varese, Italy

[4] Univ Pavia, Policlin San Matteo, Fdn Ist Ricovero & Cura Carattere Sci, I-27100 Pavia, Italy

[5] Univ Padua, Padua, Italy

[6] Univ Florence, Florence, Italy

[7] Med Univ Vienna, Inst Pathol, Vienna, Austria

[8] San Bortolo Hosp, Vicenza, Italy

[9] Mayo Clin, Rochester, MN USA

来源：

BLOOD | 2012年 / 120卷 / 03期

关键词：

WORLD-HEALTH-ORGANIZATION; MYELOPROLIFERATIVE NEOPLASMS; PROGNOSTIC-FACTORS; POLYCYTHEMIA-VERA; RISK-FACTORS; THROMBOSIS; DIAGNOSIS; SURVIVAL; CLASSIFICATION; HYDROXYUREA;

D O I：

10.1182/blood-2012-01-407981

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

In the present study, we investigated disease characteristics and clinical outcome in young patients (< 40 years) with World Health Organization (WHO)-defined essential thrombocythemia (ET) compared with early/prefibrotic primary myelofibrosis (PMF) with presenting thrombocythemia. We recruited 213 young patients (median age, 33.6 years), ncluding 178 patients (84%) with WHO-defined ET and 35 patients (16%) showing early PMF. Median follow-up time was 7.5 years. A trend for more overall thrombotic complications, particularly arterial, was seen in early PMF compared with ET. Progression to overt myelofibrosis was 3% in ET and 9% in early PMF, but no transformation into acute leukemia was observed. Combining all adverse events (thrombosis, bleeding, and myelofibrosis), the rate was significantly different (1.29% vs 3.43% of patients/year, P = .01) in WHO-ET and early PMF, respectively. In multivariate analysis, early PMF and the JAK2V617F mutation emerged as independent factors predicting cumulative adverse events. (Blood. 2012;120(3):569-571)

引用

页码：569 / 571

页数：3

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