Unmet clinical needs in the management of CALR-mutated essential thrombocythaemia: a consensus-based proposal from the European LeukemiaNet

Autor: Alvarez-Larran A, Sant'Antonio E, Harrison C, Kiladjian J, Griesshammer M, Mesa R, Ianotto J, Palandri F, Hernandez-Boluda J, Birgegard G, Nangalia J, Koschmieder S, Rumi E, Barbui T
Rok vydání: 2021
Zdroj: LANCET HAEMATOLOGY
r-INCLIVA. Repositorio Institucional de Producción Científica de INCLIVA
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ISSN: 2352-3026
Popis: Recommendations regarding management of essential thrombocythaemia rely on studi e s done before the discovery of the CALR mutation. On May 20, 2020, the Europ e a n LeukemiaNet annual meeting was held with the goal to identify unmet clin i c a l needs in myeloproliferative neoplasms. Because patients with a CALR mutation have specific clinica l characteristics, treatment of CALR-mutated essential thrombocythaemia was considered an unmet clinical need by the Europ e a n LeukemiaNet. The elaboration of a consensus document with recommendations according to current evidence was proposed as a solution for resolving uncertainties in the treatment of CALR-mutated essential thrombocythaemia. A steering committee comprising f o u r Europ e a n LeukemiaNet members was t h e n formed and a panel of ten experts in the field was recruited. T h e experts proposed 51 potential unmet clinical needs in the management of CALR-mutated essential thrombocythaemia and were as k e d to score the relevance of each topic. Those topics that obtained the highest scores as relevant unmet clinical needs were identified, including antiplat e l e t therapy in patients at low risk, definition of extreme thrombocytosis and its management in patients at low risk, indications of cytoreduction and tar g e t s of therapy, first-line treatment of choice in young patients (1500 x 109 platelets per L) with pegylated interfe r o n alfa being the preferred option for younger patients; both hydroxycarbamide and anagrelide might be given to patients ineligible for pegylated interferon alfa; and treatment algorithms for patients with high-risk pregnancies should not be changed according to genotype. The European LeukemiaNet proposes to use these recommendations in the routine management of patients wit h CALR-mutated essential thrombocythaemia, and designing new clinica l studies in this field might be useful.
Databáze: OpenAIRE
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