Calreticulin mutations and long-term survival in essential thrombocythemia

被引:89
作者
Tefferi, A. [1 ]
Wassie, E. A. [1 ]
Lasho, T. L. [1 ]
Finke, C. [1 ]
Belachew, A. A. [1 ]
Ketterling, R. P. [2 ]
Hanson, C. A. [3 ]
Pardanani, A. [1 ]
Gangat, N. [1 ]
Wolanskyj, A. P. [1 ]
机构
[1] Mayo Clin, Dept Internal Med, Div Hematol, Rochester, MN 55905 USA
[2] Mayo Clin, Div Cytogenet, Rochester, MN 55905 USA
[3] Mayo Clin, Div Hematopathol, Rochester, MN 55905 USA
关键词
WORLD-HEALTH-ORGANIZATION; INTERNATIONAL WORKING GROUP; MYELOPROLIFERATIVE NEOPLASMS; MYELOFIBROSIS RESEARCH; CALR; CLASSIFICATION; PROGNOSIS; DIAGNOSIS;
D O I
10.1038/leu.2014.148
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
The impact of calreticulin (CALR) mutations on long-term survival in essential thrombocythemia (ET) was examined in 299 patients whose diagnosis predated 2006. Mutational frequencies were 53% for Janus kinase 2 (JAK2), 32% for CALR and 3% for MPL; the remaining 12% were 'triple-negative'. We confirmed the association of mutant CALR (vs JAK2V617F) with younger age (P = 0.002), male sex (P = 0.01), higher platelet count (0.0004), lower hemoglobin (P<0.0001), lower leukocyte count (0.02) and lower incidence of recurrent thrombosis (0.04). Triple-negative patients were also younger than their JAK2-mutated counterparts (P = 0.003) and displayed lower hemoglobin (P = 0.003), lower leukocyte count (<0.0001) and lower thrombotic events (P = 0.02). Median follow-up time was 12.7 years and 47% of the patients were followed until death. Survival was the longest for triple-negative and shortest for MPL-mutated patients. Median survival was 19 years for JAK2 and 20 years for CALR-mutated cases (P = 0.32); the corresponding figures for patients of age <= 65 years were 26 and 32 years (P = 0.56). The two mutational categories were also similar for leukemic (P = 0.28) and fibrotic (P = 0.28) progression rates. The current study is uniquely characterized by its very long follow-up period and provides accurate estimates of long-term survival in ET and complements current information on mutation-specific phenotype and prognosis.
引用
收藏
页码:2300 / 2303
页数:4
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