RT Book, Section
A1 Evans, Andrew G.
A1 Friedberg, Jonathan W.
A1 Casulo, Carla
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 1180444416
T1 Burkitt Lymphoma
T2 Williams Hematology, 10e
YR 2021
FD 2021
PB McGraw-Hill Education
PP New York, NY
SN 9781260464122
LK accessmedicine.mhmedical.com/content.aspx?aid=1180444416
RD 2022/08/07
AB SUMMARYBurkitt  lymphoma  is  one  of  the  highly  aggressive  B-cell  lymphomas.  It  was  the  first  tumor  to  be  etiologically  associated  with  (a)  a  virus,  specifically  Epstein-Barr  virus,  (b)  a  specific  translocation  involving  the  MYC  oncogene,  and  (c)  one  of  the  first  cancers  shown  to  be  curable  by  chemotherapy  alone.  It  presents  in  three  clinically  distinct  forms:  endemic,  sporadic,  and  immunodeficiency  associated.  BL  is  an  uncommon  form  of  lymphoma  in  adults,  with  an  incidence  of  approximately  1200  patients  per  year  in  the  United  States.  Over  the  past  decade,  the  definition  of  BL  has  been  refined,  largely  as  a  consequence  of  improvements  in  immunohistochemical,  cytogenetic,  and  molecular  diagnostic  techniques.  Transcriptional  profiling  has  more  clearly  defined  BL  at  the  molecular  level,  and  whole-genome  sequencing  has  expanded  our  understanding  of  the  mutational  landscape  that  underlies  this  disease.  Despite  these  refinements  in  diagnostic  criteria,  the  differential  diagnosis  includes  several  aggressive  lymphomas,  including  a  group  of  patients  with  a  diagnosis  defined  by  the  World  Health  Organization  (WHO)  as  high-grade  B-cell  lymphoma  with  MYC  and  BCL2  or  BCL6  rearrangements  and  a  new  WHO  provisional  entity  called  Burkitt-like  lymphoma  with  chromosome  11q  aberration.  BL  is  a  highly  curable  malignancy  in  the  modern  therapeutic  era.  The  majority  of  younger  patients  are  cured  with  intensive  chemotherapeutic  regimens,  and  increasing  efficacy  has  been  demonstrated  with  reduced-intensity  treatments  in  older  patients.  Remaining  challenges  include  the  optimal  management  of  older  patients,  the  development  of  therapy  for  patients  with  relapsed  or  refractory  disease,  and  the  translation  of  gains  made  in  treatment  to  the  management  of  endemic  disease.