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JAK2 Inhibitors for Therapy of Myeloproliferative Neoplasms

  • Fabio P. S. Santos
  • Srdan VerstovsekEmail author
Chapter
Part of the Contemporary Hematology book series (CH)

Abstract

The classical Philadelphia chromosome-negative myeloproliferative ­neoplasms (Ph-negative MPNs) are hematopoietic stem cell disorders and include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) [1]. MF can also develop secondarily in patients with PV and ET (post-PV or -ET MF). Patients with PV and ET have close to normal life expectancy, but present with an increased risk for thrombosis [2]. They are usually treated with cytoreductive agents (e.g. hydroxyurea, anagrelide, 32P, busulphan, and pipobroman) which can effectively control elevated blood cell counts and decrease the risk of thrombotic phenomena, but may also be associated with an increased risk of transformation to acute myeloid leukemia (AML) and/or post-PV/ET MF [2–4]. Therefore, apart from hydroxyurea, there are few drugs available for treating these patients without incurring significant side effects. There are also few treatment options available for patients with MF [5]. Patients with MF suffer from different signs and symptoms, including massive splenomegaly, peripheral blood cytopenias, and constitutional symptoms such as fever, fatigue, and cachexia [5,6]. Treatment is palliative and symptom-directed.

Keywords

Polycythemia vera Essential thrombocythemia Myelofibrosis JAK2 inhibitor JAK2 V617F 

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Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  1. 1.Department of LeukemiaThe University of Texas M. D. Anderson Cancer CenterHoustonUSA
  2. 2.Department of HematologyHospital Israelita Albert EinsteinSão PauloBrazil

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