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The Clinical Management of Primary Myelofibrosis





Diagnosis and Treatment of Myelofibrosis








Managing Uncontrolled Polycythemia Vera

Myelofibrosis is a myeloproliferative neoplasm characterized by splenomegaly, progressive cytopenias, and transformation to acute myeloid leukemia. This review will describe mutations detected in myelofibrosis and discuss how to incorporate mutation information into risk stratification and therapeutic decision making for patients with myelofibrosis.

Ruben A. Mesa, MD, recently discussed the treatment considerations and decisions he makes when treating patients with polycythemia vera. Mesa, director of the University of Texas Health Cancer Center, explained his treatment decisions based on 2 case scenarios during a <em>Targeted Oncology </em>live case-based peer perspectives presentation.

Prithviraj Bose, MD, discussed the findings of 2 combination trials with ruxolitinib to optimize outcomes for patients with myelofibrosis. The JAK inhibitor ruxolitinib is the only FDA-approved therapy for the treatment of patients with myelofibrosis, but novel agents and combination regimens are in development to address some of the unmet needs in the field.

Harry P. Erba, MD, PhD, recently shared the treatment considerations and decisions he makes when treating patients with polycythemia vera.

After 4 years of follow-up, a majority of patients with polycythemia vera (PV) that responded to treatment with ruxolitinib (Jakafi) maintained their responses, results from a randomized trial showed.

Treatment with pegylated interferon alfa-2a led to objective responses in about two-thirds of patients with hydroxyurea (Hydrea)-resistant/intolerant polycythemia vera or high-risk essential thrombocytopenia, a phase II study of salvage therapy showed.

A novel pegylated formulation of interferon alfa-2b achieved a significantly higher rate of complete hematologic response compared with hydroxyurea (Hydrea) in patients with polycythemia vera, according to 2-year findings from the follow-up phase III CONTI-PV randomized trial.

The investigational activin receptor IIA ligand trap sotatercept safely increases hemoglobin levels in patients with myeloproliferative neoplasm (MPN)-associated myelofibrosis, both when used alone and in combination with ruxolitinib (Jakafi).

The investigational Nutlin family antagonist idasanutlin demonstrated significant activity and was well tolerated after multiple cycles of exposure in patients with refractory polycythemia vera.<br />






























