Significant heterogeneity in management of calreticulin-mutated essential thrombocythemia and its progression to myelofibrosis: results of a national survey

Abstract

Despite the recent publication of calreticulin (CALR)-mutated essential thrombocythemia (ET) management guidelines by the European Leukemia Net (ELN), there remains a paucity of data regarding the optimal way to manage this condition. To determine practice around Australia, we constructed a survey asking investigation and treatment questions in a hypothetical case of a young woman with CALR-mutated ET and subsequent progression to myelofibrosis. 51 of 88 hematologists replied. The responses demonstrated significant heterogeneity in specific issues such as the use of aspirin, when to initiate cytoreduction, the preferred type of cytoreduction, and platelet targets. These observations support the ELN acknowledgment that a strong evidence base for many management recommendations is lacking in this disease, and that substantial further research is needed.

Keywords:

• Calreticulin
• national survey
• myeloproliferative neoplasm

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No potential conflict of interest was reported by the author(s).

Additional information

Funding

The author(s) reported there is no funding associated with the work featured in this article.