RT Book, Section
A1 Tefferi, Ayalew
A2 Valle, David L.
A2 Antonarakis, Stylianos
A2 Ballabio, Andrea
A2 Beaudet, Arthur L.
A2 Mitchell, Grant A.
SR Print(0)
ID 1181418076
T1 The Classic Myeloproliferative Neoplasms: Chronic Myelogenous Leukemia, Polycythemia Vera, Essential Thrombocythemia, and Primary Myelofibrosis
T2 The Online Metabolic and Molecular Bases of Inherited Disease
YR 2019
FD 2019
PB McGraw-Hill Education
PP New York, NY
SN 9780071459969
LK ommbid.mhmedical.com/content.aspx?aid=1181418076
RD 2023/11/28
AB The  classic  myeloproliferative  neoplasms,  including  chronic  myelogenous  leukemia  (CML),  polycythemia  vera  (PV),  essential  thrombocythemia  (ET),  and  primary  myelofibrosis  (PMF),  are  a  phenotypically  diverse  category  of  malignancies  that  are  derived  from  stem  cells  in  the  myeloid  lineage.The  first  genetic  alteration  recognized  as  a  cause  of  myeloproliferative  disease  was  the  translocation  that  creates  the  BCR-ABL  gene  fusion.  Recently,  activating  mutations  in  JAK2  and  MPL  have  been  found  in  the  majority  of  BCR-ABL-negative  myeloproliferative  neoplasms.  Recurrent  mutations  in  these  genes  provide  clinically  useful  diagnostic  markers.Implicated  by  mutational  evidence,  the  JAK-STAT  pathway  is  a  rational  target  for  drug  development.  A  number  of  anti-JAK2  drugs  have  undergone  preclinical  testing,  and  some  of  these  have  been  introduced  into  clinical  trials.