This protocol addresses primary myelofibrosis that has progressed to accelerated or blast phase, identified by a persistent rise in blasts detected on blood smear or in the marrow, in patients who are not candidates for stem cell transplantation.
Accelerated phase is defined by a persistent increase to 10–19% blasts in blood or marrow; blast phase by a persistent increase to ≥20% blasts. Both present significant clinical challenges, particularly in older patients. This protocol applies specifically to patients who are not considered transplant eligible.
In the non-transplant setting, disease control may involve hypomethylating agents — used alone or in combination with a JAK inhibitor — or entry into a clinical trial, together with supportive care and early integration of holistic palliative care. The complete algorithm, full list of options, and sequencing criteria are available in the structured protocol.
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.
Not considered transplant eligible
HMAs, either azacytidine or decitabine, alone or combined with ruxolitinib are increasingly used regimens 'off-licence' as previous synergy has been demonstrated.
If not transplant eligible, disease control, for a period, may be achieved by clinical trial entry or HMAs with or without a JAK inhibitor. Early introduction to holistic palliative care services is advised (Grade 1C).
In the non-transplant setting, the focus is on delaying disease progression and maintaining quality of life.
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