RT Book, Section
A1 Nair, Ranjit
A1 Vega, Francisco
A1 Iyer, Swaminathan P.
A2 Kantarjian, Hagop M.
A2 Wolff, Robert A.
A2 Rieber, Alyssa G.
SR Print(0)
ID 1190833189
T1 Nodal Peripheral T-Cell Lymphoma
T2 The MD Anderson Manual of Medical Oncology, 4e
YR 2022
FD 2022
PB McGraw Hill Education
PP New York, NY
SN 9781260467642
LK hemonc.mhmedical.com/content.aspx?aid=1190833189
RD 2024/09/16
AB KEY  CONCEPTSNodal  peripheral  T-cell  lymphomas  (PTCLs)  are  categorized  into  three  basic  subtypes:  systemic  anaplastic  large-cell  lymphoma  (sALCL)  anaplastic  lymphoma  kinase  ALK  (+)  and  ALK  (-),  angioimmunoblastic  T-cell  lymphoma;  and  peripheral  T-cell  lymphoma  not  otherwise  specified  (PTCL-NOS).The  prognosis  for  patients  with  nodal  PTCLs  remains  suboptimal  compared  with  its  aggressive  B  cell  counterparts  despite  the  use  of  aggressive  chemotherapy.  The  introduction  of  novel  agents  such  as  CD30(+)  antibody–drug  conjugates  and  histone  deacetylase  inhibitors  have  brought  unprecedented  enthusiasm  in  bench  to  bedside  research  for  this  disease.It  is  recently  recognized  that  angioimmunoblastic  T–cell  lymphoma  has  its  cell  of  origin  from  CD4+  T-follicular  helper  (TFH)  cells  and  as  a  result  a  third  of  the  PTCL-NOS  are  more  aptly  diagnosed  as  angioimmunoblastic  T-cell  lymphoma  because  of  the  expression  of  TFH  markers.Understanding  of  the  genomic  landscape  has  recently  helped  to  identify  better  prognostic  markers.  Whereas  the  presence  of  ALK  or  DUSP-22  in  sALCL  is  associated  with  favorable  prognosis,  expression  of  GATA-3  or  GATA-3  target  genes  in  PTCL-NOS  is  associated  with  a  worse  prognosis.Except  for  ALK  (+)  ALCL  and  ALK  (-)  ALCL  with  DUSP22  rearrangement,  patients  with  PTCL  generally  have  a  chemoresistance,  short-term  remission,  and  early  relapse  when  treated  with  cyclophosphamide,  doxorubicin,  vincristine,  and  prednisone  (CHOP)  or  CHOP-like  chemotherapy.Combination  therapies  using  novel  agents  holds  the  future  to  improve  clinical  response  and  outcomes  in  both  frontline  and  relapsed  or  refractory  disease.  Whereas  BV-CHP  (brentuximab  vedotin  plus  cyclophosphamide,  doxorubicin,  prednisone)  is  currently  the  standard  of  care  treatment  in  patients  with  CD30(+)  PTCLs,  Cyclophosphamide,  doxorubicin,  vincristine,  and  prednisone  (CHOP),  with  or  without  the  addition  of  etoposide,  is  commonly  used  in  those  with  CD30(-)  PTCLs.