Essential thrombocythemia beyond the first decade: Life expectancy, long-term complication rates, and prognostic factors

被引:244
作者
Wolanskyj, AP
Schwager, SM
McClure, RF
Larson, DR
Tefferi, A
机构
[1] Mayo Clin, Coll Med, Div Hematol, Rochester, MN 55905 USA
[2] Mayo Clin, Coll Med, Div Hematopathol, Rochester, MN 55905 USA
[3] Mayo Clin, Coll Med, Div Biostat, Rochester, MN 55905 USA
关键词
D O I
10.4065/81.2.159
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
OBJECTIVE: To describe the long-term natural history of essential thrombocythemia (ET) in terms of life expectancy, risk of disease transformation into a more aggressive myelold disorder, and prognostic factors for both survival and disease complications. PATIENTS AND METHODS: The study population consisted of a consecutive cohort of patients seen at the Mayo Clinic in Rochester, Minn, in whom a diagnosis of ET was established before 1992, thus allowing a minimum of 10 years of potential follow-up. The conventional criteria-based diagnosis was confirmed by bone marrow biopsy in all instances. RESULTS: A total of 322 patients were studied (median age, 64 years; median follow-up, 13.6 years). With a median survival time of 18.9 years, survival in the first decade of disease was similar to that of the control population (risk ratio, 0.72; 95% confidence interval, 0.50-0.99) but became significantly worse thereafter (risk ratio, 2.21; 95% confidence interval, 1.74-2.76). Multivarlable analysis identified age at diagnosis of 60 years or older, leukocytosis, tobacco use, and diabetes mellitus as Independent predictors of poor survival. A 2-variable model based on an age cutoff of 60 years and leukocyte count of 15 x 10(9)/L resulted in 3 risk groups with significant difference In survival. In addition, age at diagnosis of 60 years or older, leukocytosis, and history of thrombosis were independent predictors of major thrombotic events. The risk of leukemic or any myeloid disease transformation was low in the first 10 years (1.4% and 9.1%, respectively) but increased substantially in the second (8.1% and 28.3%, respectively) and third (24.0% and 58.5%, respectively) decades of the disease. CONCLUSION: Life expectancy in patients with ET is significantly worse than that of the control population. Leukocytosis Is Identified as a novel Independent risk factor for both inferior survival and thrombotic events.
引用
收藏
页码:159 / 166
页数:8
相关论文
共 36 条
  • [1] Leukemic transformation of essential thrombocythemia without previous cytoreductive treatment
    Andersson, PO
    Ridell, B
    Wadenvik, H
    Kutti, J
    [J]. ANNALS OF HEMATOLOGY, 2000, 79 (01) : 40 - 42
  • [2] Barbui T, 2004, HAEMATOLOGICA, V89, P215
  • [3] Thrombosis-free survival and life expectancy in 187 consecutive patients with essential thrombocythemia
    Bazzan, M
    Tamponi, G
    Schinco, P
    Vaccarino, A
    Foli, C
    Gallone, G
    Pileri, A
    [J]. ANNALS OF HEMATOLOGY, 1999, 78 (12) : 539 - 543
  • [4] Major vascular complications in essential thrombocythemia:: a study of the predictive factors in a series of 148 patients
    Besses, C
    Cervantes, F
    Pereira, A
    Florensa, L
    Solé, F
    Hernández-Boluda, C
    Woessner, S
    Sans-Sabrafen, J
    Rozman, C
    Montserrat, E
    [J]. LEUKEMIA, 1999, 13 (02) : 150 - 154
  • [5] Myelofibrosis with myeloid metaplasia following essential thrombocythaemia:: actuarial probability, presenting characteristics and evolution in a series of 195 patients
    Cervantes, F
    Alvarez-Larrán, A
    Talarn, C
    Gómez, M
    Montserrat, E
    [J]. BRITISH JOURNAL OF HAEMATOLOGY, 2002, 118 (03) : 786 - 790
  • [6] COLOMBI M, 1991, CANCER-AM CANCER SOC, V67, P2926, DOI 10.1002/1097-0142(19910601)67:11<2926::AID-CNCR2820671136>3.0.CO
  • [7] 2-3
  • [8] HYDROXYUREA FOR PATIENTS WITH ESSENTIAL THROMBOCYTHEMIA AND A HIGH-RISK OF THROMBOSIS
    CORTELAZZO, S
    FINAZZI, G
    RUGGERI, M
    VESTRI, O
    GALLI, M
    RODEGHIERO, F
    BARBUI, T
    [J]. NEW ENGLAND JOURNAL OF MEDICINE, 1995, 332 (17) : 1132 - 1136
  • [9] INCIDENCE AND RISK-FACTORS FOR THROMBOTIC COMPLICATIONS IN A HISTORICAL COHORT OF 100 PATIENTS WITH ESSENTIAL THROMBOCYTHEMIA
    CORTELAZZO, S
    VIERO, P
    FINAZZI, G
    DEMILIO, A
    RODEGHIERO, F
    BARBUI, T
    [J]. JOURNAL OF CLINICAL ONCOLOGY, 1990, 8 (03) : 556 - 562
  • [10] Thrombosis and haemorrhage in polycythaemia vera and essential thrombocythaemia
    Elliott, MA
    Tefferi, A
    [J]. BRITISH JOURNAL OF HAEMATOLOGY, 2005, 128 (03) : 275 - 290