RT Book, Section
A1 Mehta, Neha
A1 Moskowitz, Alison
A1 Horwitz, Steven
A2 Kaushansky, Kenneth
A2 Lichtman, Marshall A.
A2 Prchal, Josef T.
A2 Levi, Marcel M.
A2 Press, Oliver W.
A2 Burns, Linda J.
A2 Caligiuri, Michael
SR Print(0)
ID 1172373755
T1 Mature T-Cell and Natural Killer Cell Lymphomas
T2 Williams Hematology, 9e
YR 2015
FD 2015
PB McGraw-Hill Education
PP New York, NY
SN 9780071833004
LK hemonc.mhmedical.com/content.aspx?aid=1172373755
RD 2024/04/19
AB SUMMARYThe  mature  T-cell  and  natural  killer  (NK)-cell  lymphomas  represent  10  to  15  percent  of  the  non-Hodgkin  lymphomas  by  incidence  and  comprise  23  clinicopathologic  entities  in  the  most  recent  classification.  They  include  cutaneous  T-cell  lymphomas,  discussed  in  Chap.  103,  and  systemic  T-cells  lymphomas,  which  are  discussed  here.  The  systemic  T-cell  lymphomas  have  highly  variable  courses  and  are  typically  aggressive  and  frequently  less  responsive  to  conventional  chemotherapy  than  their  B-cell  counterparts.  The  most  common  systemic  T-cell  and  NK-cell  lymphomas  worldwide  include  peripheral  T-cell  lymphoma,  not  otherwise  specified  (PTCL-NOS)  and  angioimmunoblastic  T-cell  lymphoma  (AITL),  representing  26  percent  and  19  percent  of  systemic  T-cell  and  NK-cell  lymphomas,  respectively.  There  is  considerable  geographic  variation  in  the  incidence  of  certain  entities,  such  as  adult  T-cell  leukemia/lymphoma  (ATL)  and  extranodal  NK/T-cell  lymphoma  (ENKTL).  In  view  of  the  rarity  of  systemic  T-cell  and  NK-cell  disorders,  large  randomized  trials  are  lacking  to  guide  therapies.  Treatment  strategies  are  generally  based  upon  the  best  data  available,  which  includes  prospective  phase  II  studies  and  retrospective  analyses.  The  most  frequently  used  regimens  for  the  more  common  entities,  PTCL-NOS,  AITL,  and  anaplastic  large  cell  lymphoma  (ALCL)  are  cyclophosphamide,  doxorubicin,  vincristine,  prednisone  (CHOP)-based,  although  long-term  outcomes  are  often  unsatisfactory.  Therefore,  ongoing  clinical  trials  are  aimed  at  improving  upon  CHOP  by  adding  novel  agents  or  using  alternate  regimens.  Although  controversial,  patients  are  often  considered  for  consolidation  with  autologous  stem  cell  transplant  in  first  remission  to  improve  remission  durations.  Recently,  targeted  agents  specific  for  particular  T-cell  and  NK-cell  lymphomas,  such  as  brentuximab  vedotin  for  ALCL  and  crizotinib  for  anaplastic  lymphoma  kinase  (ALK)-positive  ALCL,  are  now  allowing  the  investigation  of  more  individualized  therapy  for  these  entities.  Furthermore,  for  a  considerable  number  of  the  T-cell  and  NK-cell  lymphoma  entities,  including  ENKTL  and  ATL,  CHOP-based  therapy  is  ineffective,  and  treatment  strategies  are  disease-specific.  There  is  still  much  to  learn  about  the  biology  and  potential  drug  targets  for  these  diseases  and  ongoing  studies  using  gene  expression  profiling  and  genomics  may  help  answer  some  of  these  questions.  In  addition,  ongoing  clinical  trials  evaluating  disease-specific  treatment  approaches  and  employing  novel  and  often  targeted  agents  will  hopefully  lead  to  improved  outcomes  for  patients  with  these  diseases.