UPDATE ON TREATMENTS OF PERIPHERAL T CELL LYMPHOMA

3 AGGRESSIVE TYPES:
==================
1. PTLC- NOS
2. AITL: AngioImmunoblastic
3ALCL (Anaplastic large cell  (ALK +)  6% AND     alk NEGATIVE
CD30 , t(2,5)(p23,q25)
---------------------------------------------------------------------------------
1 Peripheral T cell lymphoma not otherwise specified
-PET
-Gene Profiling
treat it with EPOCH (CHOEP) or Etoposide based regimen followed by transplant
consider Romidepsin (upfront in Europe)
followed by Gemcitabine, Navelbine (Doxil)

Europeans give ACVBP +/- Romidepsin (Vindesin not available in the US)

refractory disease" FDA has released 3 drugs, their role and position is being evaluated"
=============
Romidepsin
Pralatrexate
CD30 positive - BRENTUXIMAB VEDOITIN
----------------------------------------------------------------------------------------------
2. some T cell lymphoma are clearly Indolent i.e Mycosis Fungoides (MF)
-------------------------------------------------------------------------------
3. Anaplastic Large cell Lymphoma
ALK negative
for Europeans ACVBP followed by transplant

USA, same paradigm as in PTCL NOS

FOR ALK POSITIVE -- CHOP  ALONE IS THE BEST NO TRANSPLANT (NO ETOPOSIDE NEEDED)   (THE GERMAN STUDY WAS RETROSPECTIVE )
CRIZOTINIB

PRALATREXATE AND ROMIDEPSIN HAVE BEEN USED IN CLINICAL TRIAL
---------------------------------------------------------------------------------------------------------
4. ANGIOIMMUNOBLASTIC

STEROIDS
SOME CLAIM CYCLOSPORINE (PARTICULARLY WHEN HEMOLYTIC ANEMIA)

IN EUROPE CHOP-ROMIDEPSIN

IN USA, GEMZAR
FOR THOSE WITH A COMPONENT OF CD20 POSITIVE, RITUXAN +STEROID

WHATEVER YOU DO, DON'T GIVE ADRIAMYCIN!