علاج ميلوفيبروسيس الأولي مع ارتفاع الأرومات في الدم أو النخاع: المرحلة المتسارعة ومرحلة الانفجار (المؤهلون للزرع)

References

• Accelerated phase (AP; persistent increase to 10%–19% blasts in blood or marrow) and blast phase (BP; persistent increase to ≥20% blasts in blood or marrow) MF presents many challenges, particularly in the older population.
• Considered transplant eligible
• Dependent on disease stage, kinetics, donor availability and local practice, patients often receive remission induction high-dose chemotherapy or hypomethylating agent (HMA)-based regimens with a plan to reduce blasts prior to allo-HSCT; some may proceed with sequential chemo-RIC transplant-based approaches.
• Frequently induction is with standard daunorubicin and cytarabine-based (DA) regimens and there is no direct comparison to other regimens such as fludarabine, cytarabine, granulocyte colony-stimulating factor (G-CSF) and idarubicin (FLAG-Ida), FLAG-Ida-venetoclax or liposomal cytarabine/daunorubicin (Vyxeos) in this setting.
• HMAs, either azacytidine or decitabine, alone or combined with ruxolitinib are increasingly used regimens 'off-licence' as previous synergy has been demonstrated.
• Reduction of blasts prior to transplant, in general, associates with improved outcomes and less risk of relapse (Grade 1C).