Calreticulin mutations and long-term survival in essential thrombocythemia

Ayalew Tefferi, E. A. Wassie, T. L. Lasho, C. Finke, A. A. Belachew, R. P. Ketterling, C. A. Hanson, Animesh D Pardanani, N. Gangat, A. P. Wolanskyj

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79 Scopus citations


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.Leukemia advance online publication, 30 May 2014; doi:10.1038/leu.2014.148.

Original languageEnglish (US)
StateAccepted/In press - May 5 2014

ASJC Scopus subject areas

  • Hematology
  • Anesthesiology and Pain Medicine
  • Cancer Research

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