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Myelofibrosis.

Blood 2022 November 24
The clinical phenotype of primary and post-polycythemia vera and post-essential thrombocythemia myelofibrosis (MF) is dominated by splenomegaly, symptomatology, a variety of blood cell alterations and a tendency to develop vascular complications and blast phase. Diagnosis requires to assess cell blood counts, bone marrow morphology, deep genetic evaluations and disease history. Driver molecular events consist of JAK2V617F mutation, CALR and MPL mutations, while about 8-10% of PMF are 'triple-negative'. Additional myeloid-gene variants are described in roughly 80% of patients. Currently available clinical-based and integrated clinical/molecular-based scoring systems predict survival of MF patients, and are applied for conventional treatment decision-making, indication to stem cell transplant (SCT) and allocation in clinical trials. Standard treatment consists of anemia-oriented therapies, hydroxyurea, and JAK inhibitors as ruxolitinib, fedratinib, pacritinib, momelotinib. Overall, spleen volume reduction of 35% or greater (SVR35) at week 24 can be achieved by 42% of ruxolitinib-, 47% of fedratinib-, 19% of pacritinib- and 27% of momelotinib-treated patients. Now, it is time to move from SVR35-oriented drugs to treatments with new paradigms as disease modification, that we intend as a robust and unequivocal effect on disease biology and/or on patient survival. The growing number of clinical trials potentially pave the way for new strategies in MF patients. Translational studies of some molecules showed an early effect on bone marrow fibrosis and on variant allele frequencies of myeloid genes. SCT is still the only curative option, however, associated with relevant challenges. This review focuses on diagnosis, prognostication, and treatments of MF.

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