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Jak-2 Positive Myeloproliferative Neoplasms

  • Leukemia (JP Dutcher, Section Editor)
  • Published:
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Opinion statement

Originally described by Dameshek in 1951, myeloproliferative disorders are today classified as myeloproliferative Neoplasms (MPNs) in WHO’s Classification of Tumors of Hematopoietic and Lymphoid Tissues. The term includes a range of conditions, [ie, BCR-ABL-positive chronic myelogenous leukemia (CML), chronic neutrophilic leukemia (CNL), polycythemia vera (PV), primary myelofibrosis (PMF), essential thromobocythemia (ET), chronic eosinophilic leukemia not otherwise specified (CEL-NOS), mastocytosis, and unclassifiable myeloproliferative neoplasm]. In the specific case of CML, a better understanding of the pathogenesis and pathophysiology of the disease has led to a targeted therapy. The presence of chromosome Philadelphia, t(9;22)(q34;11) results in the oncogene BCR-ABL, which characterizes the disease; this molecular rearrangement gives rise to a tyrosine-kinase, which in turn triggers the proliferation of the myeloid line through the activation of the signaling pathways downstream. Tyrosine-kinase inhibitors (TKIs) have altered the therapy and monitoring of CML patients and improved both their prognosis and quality of life. In 2005, various groups of investigators described a new point mutation of the gene JAK2 associated to MPNs. Although the presence of this mutation has led to a modification in the diagnostic criteria of these conditions, the impact of the use of JAK2 inhibitors on the prognosis and course of the disease continues to be controversial.

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Pablo J. Muxí had travel/accommodations expenses covered or reimbursed by Novartis. Ana Carolina Oliver declares that she has no conflict of interest.

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  1. Department of Haematology, British Hospital, Av. Italia 2420, Montevideo, Uruguay

    Pablo J. Muxí MD & Ana Carolina Oliver MD

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Muxí, P.J., Oliver, A.C. Jak-2 Positive Myeloproliferative Neoplasms. Curr. Treat. Options in Oncol. 15, 147–156 (2014). https://doi.org/10.1007/s11864-014-0279-3

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