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Immunotherapy and polycythemia vera


Polycythemia vera (PV) is a chronic myeloproliferative disorder characterized by abnormal growth of erythroid precursors in the bone marrow. Almost all patients with PV, around 97%, have a mutation in Janus kinase 2 (JAK2). It is through the activation of JAK/Signal Transducers and Activators of Transcription (STAT) protein signaling pathway that the JAK2 mutation is thought to induce cellular proliferation, growth, hematopoiesis and immune response in PV patients. A summary of the current role of immunotherapy in the treatment of PV is provided. There are new JAK kinase inhibitors that are currently being evaluated and are at various stages of clinical trials and development, as well as the previously studied cytokines therapies. At the present time, ruxolitinib and interferon-? (IFN-?) are the only United States Food and Drug Administration (FDA) approved drugs for the management of advanced PV. JAK kinase inhibitors are better tolerated and less problematic than the interferons. Still, allogeneic stem cell transplantation is the only potentially curable method for end-stage PV. Additional genetic mutations have been implicated in PV pathogenesis. In this perspective, targeting different pathways might be required. Further investigations are needed to evaluate the promising role of immunotherapy in PV whether alone or in combination with other modalities.

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