RT Book, Section
A1 Larson, Richard A.
A2 Kaushansky, Kenneth
A2 Prchal, Josef T.
A2 Burns, Linda J.
A2 Lichtman, Marshall A.
A2 Levi, Marcel
A2 Linch, David C.
SR Print(0)
ID 1180447317
T1 Acute Lymphoblastic Leukemia
T2 Williams Hematology, 10e
YR 2021
FD 2021
PB McGraw-Hill Education
PP New York, NY
SN 9781260464122
LK hemonc.mhmedical.com/content.aspx?aid=1180447317
RD 2024/04/16
AB SUMMARYAcute  lymphoblastic  leukemia  (ALL)  is  a  malignant  disorder  that  originates  in  a  single  B-  or  T-lymphocyte  progenitor.  Proliferation  and  accumulation  of  clonal  blast  cells  in  the  marrow  result  in  suppression  of  hematopoiesis  and,  thereafter,  anemia,  thrombocytopenia,  and  neutropenia.  Lymphoblasts  can  accumulate  in  various  extramedullary  sites,  especially  the  meninges,  gonads,  thymus,  liver,  spleen,  and  lymph  nodes.  The  disease  is  most  common  in  children  but  occurs  in  individuals  of  any  age.  ALL  has  many  subtypes  and  can  be  classified  by  immunologic,  cytogenetic,  and  molecular  genetic  methods.  These  methods  identify  clinically  important,  biologic  subtypes  requiring  treatment  approaches  that  differ  in  their  use  of  specific  drugs  or  drug  combinations,  dosages  of  drug,  or  duration  of  treatment  required  to  achieve  optimal  results.  For  example,  cases  of  childhood  ALL  having  a  hyperdiploid  karyotype  respond  well  to  extended  treatment  with  methotrexate  and  mercaptopurine,  whereas  adults  whose  leukemic  cells  contain  the  Philadelphia  chromosome  and  BCR-ABL1  fusion  benefit  from  treatment  that  includes  a  tyrosine  kinase  inhibitor  and  transplantation  of  allogeneic  hematopoietic  stem  cells.  The  lesser  rate  of  therapeutic  success  in  adult  ALL  is  partly  related  to  a  high  frequency  of  cases  having  unfavorable  genetic  abnormalities  and  partly  related  to  poor  tolerance  for  intensive  treatment.  Nearly  90%  of  children  and  40%  of  adults  can  expect  long-term,  leukemia-free  survival—and  probable  cure—with  contemporary  treatment.  Novel  immunotherapeutic  approaches  are  under  development.  Currently,  emphasis  is  placed  not  only  on  improving  the  cure  rate  but  also  on  improving  quality  of  life  by  preventing  acute  and  late  treatment–related  complications,  such  as  second  malignancies,  cardiotoxicity,  and  endocrinopathy.