RT Book, Section
A1 Kantarjian, Hagop
A1 Cortes, Jorge
A2 Longo, Dan L.
SR Print(0)
ID 1135227203
T1 Chronic Myeloid Leukemia
T2 Harrison's Hematology and Oncology, 3e
YR 2016
FD 2016
PB McGraw-Hill Education
PP New York, NY
SN 9781259835834
LK hemonc.mhmedical.com/content.aspx?aid=1135227203
RD 2024/04/19
AB Chronic  myeloid  leukemia  (CML)  is  a  clonal  hematopoietic  stem  cell  disorder.  The  disease  is  driven  by  the  BCR-ABL1  chimeric  gene  product,  a  constitutively  active  tyrosine  kinase,  resulting  from  a  reciprocal  balanced  translocation  between  the  long  arms  of  chromosomes  9  and  22,  t(9;22)  (q34;q11.2),  cytogenetically  detected  as  the  Philadelphia  chromosome  (Ph)  (Fig.  15-1).  Untreated,  the  course  of  CML  may  be  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  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  but  risky  treatment  approach,  is  now  offered  as  second-  or  third-line  therapy  after  failure  of  TKIs.