CALR-Mutated Essential Thrombocythemia Associated With Higher Progression to Myelofibrosis Risk

CALR mutations in essential thrombocythemia are associated with lower thrombosis risk and higher risk of progression to myelofibrosis (MF) compared with other driver mutations, according to a recent study.

The study was led by Katie Erdos, a Research Program Assistant at Weill Cornell Medicine’s Richard T. Silver, MD Myeloproliferative Neoplasms Center, and presented at the 65th American Society of Hematology Annual Meeting & Exposition.

Erdos and colleagues conducted the study to evaluate the impact of driver mutations on the risks of thromboembolic events, disease progression, and patient mortality.

Of 338 total patients, 216 (64%) were positive for JAK2V617F, 85 (25%) were positive for CALR, 19 (6%) were positive for MPL, and 18 were (5%) triple-negative (TN). Red cell parameters were slightly higher in patients with JAK2V617F mutations (P<0.001), white blood cell count was highest in TN patients (P=0.012), and platelet count did not significantly vary across mutation groups (P=0.064).

The 20-year thrombosis-free survival was 71% for JAK2V617F, 100% for CALR, 90% for MPL, and 83% for TN (P=0.0027). The 20-year MF-free survival was 87% for JAK2V617F, 48% for CALR, 65% for MPL, and 94% for TN (P=0.00053). Meanwhile, the 20-year overall survival was 76% for JAK2V617F, 86% for CALR, 89% for MPL, and 90% for TN (P=0.66).

“Our findings reinforce the need for long-term data to guide therapy for ET based not only on the near-term thrombotic risk, but also on the long-term risk of progression,” wrote Erdos and colleagues.

Reference

Erdos K, Lee N, Lebbe A, et al. Low thrombosis risk CALR mutations confer higher risk of essential thrombocythemia progression. Abstract #1819. Presented at the 65th ASH Annual Meeting & Exposition; December 9-12, 2023; San Diego, California.

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Posted in Essential Thrombocythemia.

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