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Eur J Haematol. 2015 Jan;94(1):31-6. doi: 10.1111/ejh.12389. Epub 2014 Dec 4.

Mutations and thrombosis in essential thrombocythemia: prognostic interaction with age and thrombosis history.

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1
Division of Hematology, Mayo Clinic, Rochester, MN, USA.

Abstract

BACKGROUND:

Vascular events in essential thrombocythemia (ET) are associated with advanced age and thrombosis history. Recent information suggests additional effect from the presence of specific mutations.

OBJECTIVES:

To examine the influence of age and thrombosis history on the reported association between mutational status and thrombosis-free survival in ET.

PATIENTS AND METHODS:

Analysis was performed using a Mayo Clinic cohort of 300 ET patients, and key findings were reanalyzed by including additional 102 Italian patients.

RESULTS:

Among 300 Mayo patients with ET (median age 55 yr, 60% females), mutational frequencies were 53% JAK2, 32% CALR, 3% MPL, and 12% JAK2, CALR and MPL wild type. One hundred and six (35%) patients experienced arterial (n = 75) or venous (n = 43) events, before (n = 55) or after (n = 71) diagnosis. In univariate analysis, compared to JAK2-mutated cases, JAK2, CALR and MPL wild type (HR 0.31, 95% CI 0.11-0.86), and CALR-mutated (0.53, 95% CI 0.30-0.92) patients displayed better thrombosis-free survival. JAK2, CALR, and MPL wild type remained significant (P = 0.03; HR 0.32, 95% CI 0.11-0.9) during multivariable analysis that included age (P = 0.01) and thrombosis history (P = 0.0006); a favorable impact from CALR mutations was of borderline significance (P = 0.1; HR 0.62, 95% CI 0.35-1.1), but became significant (P = 0.02) when multivariable analysis including thrombosis history (P = 0.02) was performed on patients younger than 60 yr of age.

CONCLUSIONS:

The favorable impact of mutational status on thrombosis-free survival in ET might be most evident for JAK2, CALR, and MPL wild type patients, whereas the favorable effect from CALR mutations might be confined to young patients.

KEYWORDS:

calreticulin; essential thrombocythemia; janus kinase 2; mutation; thrombosis

Comment in

PMID:
24889737
DOI:
10.1111/ejh.12389
[Indexed for MEDLINE]
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