Indolent TCL facts

Question 1

MF Tx of limited disease

Answer 1

Topical steroids
Topical mustard nitrogen
Radiotherarpy

Question 2

MF Tx of widespread skin disease

Answer 2

Phototherapy ultraviolet A/B + psoralen
Bv
SS pts were excluded from ALCANZA trial of Bv in MF

Question 3

MF immunophenotype

Answer 3

Cd3+, CD4+, CD8-, CD30 +/-,
aberrant loss of 1 of:
CD2, C5, CD7

Question 4

TX of advanced MF/SS with CD30+

Answer 4

BV if CD30+
Mogamulizumab (anti- CCR4) if CD30-

Question 5

Agents active in MF/SS

Answer 5

Romidepsin
Lenalidomide
Mogamulizumab (CCR4)
Alemtuzumab (CD52)
Bv

Question 6

Consolidation Tx for SS

Answer 6

Auto or Allo trasplant

Question 7

Cutaneous ALCL Tx

Answer 7

if localized- RT

Question 8

Breast implant–associated ALCL

Answer 8

CD30+
ALK-
Tx is surgical removal of implants
if more nodal disease- Tx like RT or Bv-CHP

Question 9

T-LGL immunophenotype

Answer 9

CD3 and CD8 positive
CD57/CD16 positive
CD56 negative

3+57=60
8+16=24

Question 10

NK-LGL immunophenotype

Answer 10

CD2+, CD8+,CD16+. CD56+
sCD3− , CD4-, CD57-

Question 11

LGLL Tx

Answer 11

MTX/CY/CsA
+/- prednisone
2nd line- alemtuzumab
Consider splenectomy

Question 12

LGLL driver mutation

Answer 12

STAT3