Anagrelide and the CALR mutation allele burden in essential thrombocythemia
Atkinson, E.; Bakri, M.; Hayat, A.; Langabeer, S.E.
Experimental Oncology 40(2): 152-153
2018
ISSN/ISBN: 1812-9269 PMID: 29949532 Document Number: 7111
Despite anagrelide therapy, no impact on the CALR mutation allele burden was evident in this instance. While some emerging evidence suggests that driver mutation status may impact on the clinical response, the above observation is broadly in line with the finding that in essential thrombocythemia, anagrelide therapy also does not impact on the JAK2 V617F allele burden. While interferon and JAK½ inhibitors are known to exert some of their effects through disruption of immune responses, the full mechanism of action of anagrelide remains unclear with the primary effect being inhibition of megakaryocyte maturation and proplatelet formation. This inability to directly target the malignant clone may reflect the inability of anagrelide to induce molecular responses in essential thrombocythemia, if indeed deep responses are required for long-term survival. This observation requires confirmation in independent patient cohorts.
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