RT Book, Section
A1 Geskin, Larisa J.
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 1121100771
T1 Cutaneous T-Cell Lymphoma (Mycosis Fungoides and SéZary Syndrome)
T2 Williams Hematology, 9e
YR 2015
FD 2015
PB McGraw-Hill Education
PP New York, NY
SN 9780071833004
LK accessmedicine.mhmedical.com/content.aspx?aid=1121100771
RD 2024/04/19
AB SUMMARYCutaneous  T-cell  lymphoma  (CTCL)  is  a  heterogeneous  group  of  malignant  lymphomas  that  share  the  propensity  for  malignant  T  lymphocytes  expressing  cutaneous  lymphocyte  antigen  to  infiltrate  the  skin.  Mycosis  fungoides  (MF)  is  the  most  common  variant  of  CTCL,  representing  approximately  50  percent  of  all  cases.  Sézary  syndrome  (SS)  is  a  leukemic  variant  of  MF,  affecting  approximately  5  percent  of  patients  with  MF.1  MF  and  SS  are  the  most  common  malignant  proliferations  of  mature  memory  T  lymphocytes  of  the  helper  phenotype  (CD4+CD45RO+),2  which  renders  patients  immunocompromised  even  at  the  earliest  stages  of  the  disease.  Advanced  stages  are  associated  with  severe  immune  suppression.  Diagnosis  is  established  by  skin  biopsy,  followed  by  staging  work  up  which  includes  radiologic  imaging  and  pathologic  evaluation  of  the  lymph  nodes,  internal  organs,  blood,  and  marrow,  as  appropriate,  according  to  presenting  manifestations  of  the  disease.MF  is  divided  into  early  and  advanced  stages  for  therapeutic  and  prognostic  reasons.  In  early  stages,  the  disease  follows  an  indolent  course  and  has  a  favorable  prognosis.  In  advanced  stages,  the  prognosis  is  poor.  There  are  numerous  therapeutic  options.  No  treatment  has  been  definitively  proven  to  improve  survival,  but  newer  studies  suggest  that  survival  is  longer  than  historically  documented.3,4  Multiagent  chemotherapy  is  not  a  useful  option  because  it  is  inferior  to  immune  modulating  and  biologic  therapies.5  Considering  the  overall  protracted  course  of  the  disease,  its  indolent  character,  immunocompromised  status  of  the  patients,  and  absence  of  definitive  therapy,  aggressive  multiagent  chemotherapy  contributing  to  immunosuppression  should  be  reserved  for  end-stage  palliation  or  as  a  bridge  to  stem  cell  transplantation  with  the  goal  of  definitive  cure.6,7,8  There  are  several  FDA-approved  single  agents  for  therapy  of  MF,  which  are  safe  and  effective  against  the  disease  and  may  be  used  in  the  therapeutic  ladder  prior  to  multi-agent  regimens.9,10,11  Several  single  agents  and  combinations  of  new  and  older  agents  are  in  clinical  trials  now  to  test  for  efficacy  in  MF  and  SS.  Because  no  single  therapy  is  considered  to  be  the  standard  of  care  for  MF  and  SS,  clinical  trials  remain  a  viable  option  for  most  patients.  The  goal  of  therapy  is  to  induce  long-term  remissions  without  compromising  patients’  immunity  and  improvement  of  the  quality  of  life.