Idiopathic myelofibrosis (IMF; also referred to as agnogenic myeloid metaplasia or myelosclerosis with myeloid metaplasia) is a chronic myeloproliferative disorder characterized by bone marrow fibrosis secondary to cytokine release from a clonal proliferation of hematopoietic stem cells and resulting in extramedullary hemopoiesis. Myeloablative allogeneic stem cell transplantation (allo-SCT) is the only curative treatment for myelofibrosis, although it is associated with high transplant-related mortality (TRM), particularly in the elderly, making most patients with IMF ineligible for this treatment.
The results of published studies strongly support allo-SCT as an effective treatment strategy in patients with IMF, indicating that it has moved from the experimental setting into that of a practical therapeutic option. Despite the potential for cure, the use of myeloablative protocols is hampered by the advanced age of most patients at diagnosis, and the high TRM associated with this procedure. Based on the available evidence, myeloablative transplantation should only be considered for younger patients.
The NCI lists allogeneic transplantation as an option for this disease. The literature generally supports it for patients with a better prognosis, earlier disease and better performance status.
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