Refined cytogenetic-risk categorization for overall and leukemia-free survival in primary myelofibrosis: a single center study of 433 patients

被引:149
作者
Caramazza, D. [2 ]
Begna, K. H. [1 ]
Gangat, N. [1 ]
Vaidya, R. [1 ]
Siragusa, S. [2 ]
Van Dyke, D. L. [3 ]
Hanson, C. [4 ]
Pardanani, A. [1 ]
Tefferi, A. [1 ]
机构
[1] Mayo Clin, Div Hematol, Dept Med, Rochester, MN 55905 USA
[2] Policlin Univ Palermo, Cattedra & UO Ematol, Palermo, Italy
[3] Mayo Clin, Cytogenet Div, Dept Lab Med, Rochester, MN 55905 USA
[4] Mayo Clin, Div Hematopathol, Dept Lab Med, Rochester, MN 55905 USA
关键词
myelofibrosis; karyotype; cytogenetics; prognosis; myeloproliferative; INTERNATIONAL WORKING GROUP; PROGNOSTIC SCORING SYSTEM; POLYCYTHEMIA-VERA; MYELOPROLIFERATIVE NEOPLASMS; ESSENTIAL THROMBOCYTHEMIA; MYELOID METAPLASIA; ALLELE BURDEN; JAK2V617F; DISORDERS; DISEASE;
D O I
10.1038/leu.2010.234
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
We have previously identified sole +9, 13q- or 20q-, as 'favorable' and sole +8 or complex karyotype as 'unfavorable' cytogenetic abnormalities in primary myelofibrosis (PMF). In this study of 433 PMF patients, we describe additional sole abnormalities with favorable (chromosome 1 translocations/duplications) or unfavorable (-7/7q-) prognosis and also show that other sole or two abnormalities that do not include i(17q), -5/5q-, 12p-, inv(3) or 11q23 rearrangement are prognostically aligned with normal karyotype, which is prognostically favorable. These findings were incorporated into a refined two-tired cytogenetic-risk stratification: unfavorable and favorable karyotype. The respective 5-year survival rates were 8 and 51% (hazard ratio (HR): 3.1, 95% confidence interval (CI): 2.2-4.3; P<0.0001). Multivariable analysis confirmed the International Prognostic Scoring System (IPSS)-independent prognostic value of cytogenetic-risk categorization and also identified thrombocytopenia (platelets <100 x 10(9)/l) as another independent predictor of inferior survival (P<0.0001). A similar multi-variable analysis showed that karyotype (P = 0.001) and platelet count (P = 0.04), but not IPSS (P = 0.27), predicted leukemia-free survival; the 5-year leukemic transformation rates for unfavorable versus favorable karyotype were 46 and 7% (HR: 5.5, 95% CI: 2.5-12.0; P<0.0001). This study provides the rationale and necessary details for incorporating cytogenetic findings and platelet count in future prognostic models for PMF. Leukemia (2011) 25, 82-88; doi: 10.1038/leu.2010.234; published online 14 October 2010
引用
收藏
页码:82 / 88
页数:7
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