Myeloproliferative and lymphoproliferative disorders: State of the art |
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Authors: | Elisa Rumi Claudia Baratè Giulia Benevolo Margherita Maffioli Alessandra Ricco Emanuela Sant'Antonio |
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Affiliation: | 1. Department of Molecular Medicine, University of Pavia, Pavia, Italy;2. Department of Clinical and Experimental Medicine, Section of Hematology, University of Pisa, Pisa, Italy;3. Hematology, Città della Salute e della Scienza, Turin, Italy;4. Hematology Unit, Ospedale di Circolo, ASST Sette Laghi, Varese, Italy;5. Department of Emergency and Organ Transplantation (D.E.T.O), Hematology Section, University of Bari, Bari, Italy;6. UOC Ematologia Aziendale, Azienda Usl Toscana Nord Ovest, Pisa, Italy Medical Genetics, University of Siena, Siena, Italy |
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Abstract: | Myeloproliferative neoplasms (MPNs), including polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF), are clonal disorders complicated mainly by vascular events and transformation to myelofibrosis (for PV and ET) or leukemia. Although secondary malignancies, in particular, lymphoproliferative disorders (LPNs), are rare, they occur at a higher frequency than found in the general population, and there has been recent scientific discussion regarding a hypothetical relationship between treatment with JAK inhibitors in MPN and the risk of development of LPN. This has prompted increased interest regarding the coexistence of MPN and LPN. This review focuses on the role of JAK2 and the JAK/STAT pathway in MPN and LPN, whether there is a role for the genetic background in the occurrence of both MPN and LPN and whether there is a role for cytoreductive drugs in the occurrence of both MPN and LPN. Furthermore, whether an increased risk of lymphoma development is limited to patients who receive the JAK inhibitor ruxolitinib, is a more general phenomenon that occurs following JAK1/2 inhibition or is associated with preferential JAK1 or JAK2 targeting is discussed. |
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Keywords: | JAK inhibitors JAK/STAT pathway JAK2 lymphoproliferative disorders myeloproliferative neoplasms ruxolitinib |
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