PTCL facts Flashcards
ATL pathogen
HTLV1
usually acquired in birth/infancy
with high viral load
ATL median OS
8-10 months
Immunophenotype of ATL
CD2, CD3, CD4, CD5- positive
BCL2) MUM1, TCR b, PD1, CD25- positive
CD20, BCL5, CD10, CD21, CD23, CD30, and cyclin D1- negative
Ki67-90-100%
Subtypes of ATL
Acute
Chronic
Smoldering
Lymphoma
ABx prophylaxis in ATL
Acyclovir, cotrimoxazole, and fluconazole
Raltegravir in ATL
Integrase inhibitor
effective in HTLV1
given prior to allo transplant to prevent new infection of graft
Mogamulizumab Tx in ATL
CCR4 antibody
increases risk for GVHD post transplant
effective but not in 1st line
Tx options for transplant ineligible pts with ATL
ZDV/IFNa maintenance
Tx of ATCL- lymphoma subtype
Bv-CHP/DA-EPOCH
Allo transplant
IT MTX
Tx of ATCL- acute leukemia subtype
ZDV/IFNa
ZDV
Zidovudine
used in HIV
nucleoside reverse transcriptase inhibitor
Tx of ATL -HTLV1+
Most common PTCL subtypes
PTCL-NOS
AITL
ALCL (ALK+-)
NKTCL
ATL
Romidepsin in PTCL
No advantage of ro-CHOP over CHOP
But signal for benefit in follicular helper subtype
Romi is active in RR setting. Mainly TFH.
Approved 2nd line Tx in PTCL
Romidepsin- HDACi
Belinostat- HDACi
Chidamide- HDACi
Pralatrexate- DHFRi
Bv
Forodesine- PNPI
PTCL-NOS subtypes (gene expression)
GATA3- poor
TBX-21- favorable
TFH PTCL subtypes
Angioimmunblastic
Nodal TFH cell lymphoma, follicular type
Nodal TFH cell lymphoma, NOS
Options in 2nd line TFH PTCL
Romidepsin
Lenalidomide
Duvelisib
Options in 2nd line PTCL-NOS
Duvelisib
Pralatrexate
Consolidation of PTCL
Auto transplant in CR1
Allo transplant in CR2 or in CR1 for very aggressive subtypes
ALK+ ALCL and ALK neg DUSP22- no consolidation
ALK- ALCL pts not nedding ASCT
DUSP22
2nd line Tx of ALK+ ALCL
ALK i
Romidepsin in RR PTCL
May be effective in TFH
Better to combine with AZA/PI3Ki
Tx of advanced MF
Bv if CD30+
Otherwise- Romidepsin, Vorinostat, Mogamolizumab
Consolidation of MF/SS
Allo
No role for auto
