Current Treatment Options in Oncology

, Volume 15, Issue 2, pp 147–156

Jak-2 Positive Myeloproliferative Neoplasms

Leukemia (JP Dutcher, Section Editor)

DOI: 10.1007/s11864-014-0279-3

Cite this article as:
Muxí, P.J. & Oliver, A.C. Curr. Treat. Options in Oncol. (2014) 15: 147. doi:10.1007/s11864-014-0279-3

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.

Keywords

Myeloproliferative neoplasmsChronic myelogenous meukemia (CML)Chronic neutrophilic leukemia (CNL)Polycythemia vera (PV)Primary myelofibrosis (PMF)Essential thromobocythemiaJAK2JAK2 inhibitorsRuxolitinib

Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  1. 1.Department of HaematologyBritish HospitalMontevideoUruguay