RT Book, Section
A1 Kantarjian, Hagop
A1 Cortes, Jorge
A2 Jameson, J. Larry
A2 Fauci, Anthony S.
A2 Kasper, Dennis L.
A2 Hauser, Stephen L.
A2 Longo, Dan L.
A2 Loscalzo, Joseph
SR Print(0)
ID 1156754453
T1 Chronic Myeloid Leukemia
T2 Harrison's Principles of Internal Medicine, 20e
YR 2018
FD 2018
PB McGraw-Hill Education
PP New York, NY
SN 9781259644016
LK accesspharmacy.mhmedical.com/content.aspx?aid=1156754453
RD 2024/04/20
AB Chronic  myeloid  leukemia  (CML)  is  a  clonal  hematopoietic  stem  cell  disorder.  The  disease  is  driven  by  the  BCR-ABL1  chimeric  gene  product,  that  codes  for  a  constitutively  active  tyrosine  kinase,  resulting  from  a  reciprocal  balanced  translocation  between  the  long  arms  of  chromosomes  9  and  22,  t(9;22)(q34.1;q11.2),  known  as  the  Philadelphia  chromosome  (Ph)  (Fig.  101-1).  Untreated,  the  course  of  CML  is  typically  biphasic  or  triphasic,  with  an  early  indolent  or  chronic  phase,  followed  often  by  an  accelerated  phase  and  a  terminal  blastic  phase.  Before  the  era  of  selective  BCR-ABL1  tyrosine  kinase  inhibitors  (TKIs),  the  median  survival  in  CML  was  3–7  years,  and  the  10-year  survival  rate  was  30%  or  less.  Introduced  into  standard  CML  therapy  in  2000,  TKIs  have  revolutionized  the  treatment,  natural  history,  and  prognosis  of  CML.  Today,  the  estimated  10-year  survival  rate  with  imatinib  mesylate,  the  first  BCR-ABL1  TKI  approved,  is  85%.  Allogeneic  stem  cell  transplantation  (SCT),  a  curative  approach  but  one  that  involves  more  risks,  is  now  more  often  offered  as  second-  or  third-line  therapy  after  failure  of  TKIs.