RT Book, Section
A1 Dickinson, Michael
A1 Seymour, John F.
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 1180448123
T1 Diffuse Large B-Cell Lymphoma and Related Diseases
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=1180448123
RD 2024/04/18
AB SUMMARYDiffuse  large  B-cell  lymphoma  (DLBCL)  is  the  most  common  lymphoma  diagnosis  globally.  The  term  DLBCL  encompasses  several  distinct  subtypes  of  lymphoma  that  differ  by  histologic  features,  molecular  drivers,  epidemiologic  risk  factors,  and  at  times,  treatment  selection.  The  common  unifying  feature  is  the  presence  of  aggregates  of  large,  malignant  centroblast  B  lymphocytes.  Although  DLBCL  occurs  at  all  ages,  the  incidence  increases  with  age  and  peaks  in  the  seventh  decade  of  life.  The  typical  clinical  presentation  is  with  a  rapidly  enlarging  lymph  nodes,  although  extranodal  involvement  occurs  frequently.  Systemic  symptoms  occur  in  a  substantial  subset  of  patients  and  include  fever,  sweats,  and  weight  loss.  The  goal  of  treatment  is  cure,  which  is  achievable  in  approximately  two-thirds  of  patients  with  combination  chemoimmunotherapy.  The  mainstay  of  therapy  is  cyclophosphamide,  doxorubicin,  vincristine,  prednisolone,  and  rituximab  (R-CHOP)  given  every  21  days  for  six  cycles.  Early-stage  disease  may  also  be  managed  with  fewer  cycles  of  chemoimmunotherapy  with  or  without  radiotherapy.  In  patients  who  relapse,  salvage  chemotherapy  at  standard  doses  is  rarely  curative.  High-dose  chemotherapy  and  autologous  stem  cell  transplantation  cure  a  significant  minority  of  patients  who  have  relapsed  disease.  Chimeric  antigen  receptor  T  cells  targeting  the  cluster  of  differentiation-19  (CD19)  antigen  have  efficacy  as  a  third-line  or  subsequent  therapy  and  are  being  evaluated  as  an  alternative  to  autologous  stem  cell  transplantation  in  first  relapse  in  prospective  clinical  trials.