PTCL facts

Question 1

ATL pathogen

Answer 1

HTLV1
usually acquired in birth/infancy
with high viral load

Question 2

ATL median OS

Answer 2

8-10 months

Question 3

Immunophenotype of ATL

Answer 3

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%

Question 4

Subtypes of ATL

Answer 4

Acute
Lymphoma
Chronic
Smoldering

Question 5

ABx prophylaxis in ATL

Answer 5

Acyclovir, cotrimoxazole, and fluconazole

Question 6

Raltegravir in ATL

Answer 6

Integrase inhibitor
effective in HTLV1
given prior to allo transplant to prevent new infection of graft

Question 7

Mogamulizumab on ATL

Answer 7

CCR4 antibody
increases risk for GVHD post transplant
effective but not in 1st line

Question 8

Tx options for tranplant ineligible pts with ATL

Answer 8

ZDV/IFNa maintenance

Question 9

Tx of ATCL- lymphoma subtype

Answer 9

Bv-CHP/DA-EPOCH
Allo transplant
IT MTX

Question 10

Tx of ATCL- acute leukemia subtype

Answer 10

ZDV/IFNa

Question 11

ZDV

Answer 11

Zidovudine
used in HIV
nucleoside reverse transcriptase inhibitor
Tx of ATL -HTLV1+

Question 12

most common PTCL subtypes

Answer 12

PTCL-NOS
AITL
ALCL (ALK+-)
NKTCL
ATL

Question 13

Romidepsin in PTCL

Answer 13

No advantage of ro-CHOP iver CHOP

Question 14

Approved 2nd line Tx in PTCL

Answer 14

Romidepsin- HDACi
Belinostat- HDACi
Chidamide- HDACi
Pralatrexate- DHFRi
Bv
Forodesine- PNPI

Question 15

PTCL-NOS subtypes (gene expression)

Answer 15

GATA3
TBX-21

Question 16

TFH PTCL subtypes

Answer 16

Angioimmunblastic
Nodal TFH cell lymphoma, follicular type
Nodal TFH cell lymphoma, NOS

Question 17

options in 2nd line TFH PTCL

Answer 17

Romidepsin
Lenalidomide
Duvelisib

Question 18

options in 2nd line PTCL-NOS

Answer 18

Duvelisib
Pralatrexate

Question 19

Consolidation of PTCL

Answer 19

Auto transplant in CR1
Allo transplant in CR2