Annals of hematology · 2025
Impact of calreticulin mutations on treatment and survival outcomes in myelofibrosis during ruxolitinib therapy.
СтатьяМногоцентровое исследованиеНаблюдательное исследованиеНазвание и аннотация: Перевод в очереди
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Palandri Francesca, Branzanti Filippo, Morsia Erika, Dedola Alessandra, Benevolo Giulia, Tiribelli Mario, Beggiato Eloise, Farina Mirko, Martino Bruno, Caocci Giovanni, Pugliese Novella, Tieghi Alessia, Crugnola Monica, Binotto Gianni, Cavazzini Francesco, Abruzzese Elisabetta, Isidori Alessandro, Scalzulli Emilia, D'Agostino Domenico, Caserta Santino, Nardo Antonella, Lemoli Roberto Massimo, Cilloni Daniela, Bocchia Monica, Pane Fabrizio, Heidel Florian H, Palumbo Giuseppe A, Breccia Massimo, Elli Elena M, Bonifacio Massimiliano
Аннотация
Calreticulin (CALR) mutations are detected in around 20% of patients with primary and post-essential thrombocythemia myelofibrosis (MF). Regardless of driver mutations, patients with splenomegaly and symptoms are generally treated with JAK2-inhibitors, most commonly ruxolitinib. Recently, new therapies specifically targeting the CALR mutant clone have entered clinical investigation. To collect information on efficacy and safety of ruxolitinib in CALR-mutated patients, we report a sub-analysis of the "RUX-MF" (NCT06516406) study, comprising 135 CALR-mutated and 786 JAK2-mutated ruxolitinib-treated patients. Compared to JAK2-mutated patients, CALR-mutated patients started ruxolitinib with a more severe disease (higher peripheral blast counts, lower hemoglobin levels and worse marrow fibrosis) and after a longer median time from diagnosis (2.6 versus 0.7 years, p < 0.001). At 6 months, spleen responses were numerically inferior in CALR-mutated patients, who also had significantly lower rates of symptom responses (56.1% versus 66.7%, p = 0.04).