TLPDs

Question 1

Subtypes of TLPDs

Answer 1

I. Nodal (Aggressive):
* PTCL, NOS
* Nodal TFH
* Anaplastic LCL
* Nodal EBV+ T/NK

II. Extranodal:
* EnNKTL
* Primary cutaneous
* HSTL
* Intestinal TCL

III. Leukaemic:
* Indolent:
* LGL (T&NK)
* Aggressive
* TPLL
* Sezary s/d
* ANKL
* ATLL

Question 2

Features of LGL

Answer 2

• Lymphos 2-20 OR > 50%
• Anaemia (PRCA) & Neutropenia
• HSM (rarely LAD)
• Associated with AI d/o (RA)
• HTLV-1

Question 3

IMPT of LGL

Answer 3

I. TLGL:
* alpha-beta Ts
* Positive for CD3, CD8, CD2 & CD57
* Negative for CD5, CD7 and CD56

II. NK-LGL:
* Positive for CD16 and CD56
* Negative for CD3

Question 4

Morphology of LGL

Answer 4

• LGL with abundant cytoplasm
• Varied cellularity
• MF
• No neoplstic Lymphoid nodules
• Intertitial/Intrasinusoidal

Question 6

Treatment of LGL

Answer 6

• Combination Chemo
  
  • MTX
  • cyclophos/cyclosporin
  • C/S
• Splenectomy
• RR = Purin analogues (Nelarabine)

Question 7

Features of TPLL

Answer 7

• +++ WCC, HSM&LAD, < Hb and Plts
• Blebs
• inv14
• IMPT: CD4 with CD52
• Tx: Alemtuzumab (Anti-CD52)

Question 8

Clinical triad and IMPT of Sezary s/d

Answer 8

1. Erythroderma
2. LAD
3. Sezary Cells (alpha-beta CD4 and CD25+ Ts)

Note:
* SS count >1000/uL
OR
* CD4:CD8 >10
* CD4+ CD7- >40%
* CD4+ CD26- >30%
* ?Of T-cells/lymphos

Question 9

Treatment of SS

Answer 9

I. Skin directed:
* Topical:
* C/S
* Topical chemo
* Procedural:
* PhotoTx
* RT
* ECP (Extracorporeal photopheresis)

II. Systemic Tx:
* Chemo (CHOP)
* ImmuTx (IFNa)
* Oral retinoids
* Mabs and BV
* AlloHSCT

Question 10

Features of Aggressive NKcell Lymphoma

Answer 10

• LAD, HSM, sick and leukaemic
• Positive for CD8, CD2, CD7, CD15 and CD56
• Ample pale granular cytoplasm and haemophagocytosis
• EBV

Question 11

Features of Adult T-cell Leukaemia/Lymphoma

Answer 11

• HTLV-1
• Pleomorphic = Flower/HRS-like
• Normal counterpart = CD4+, CD25+ FOXP3 Tregs
• CD4>CD8
• Positive for CD2, CD3, CD5, CD25 ± CD30 AND Negative for CD7

Variants:
* Acute (Aggr)
* Leukaemic
* Lymphoma
* Chronic
* Unfavourable (Aggr)
* Favourable (Indol)
* Smouldering (Indol)

Question 12

Features of Anaplasic LCL

Answer 12

• Sheets of pleomorphic/CD30+ large cells
• Often no T-markers
• ALK+, ALK-, Breast-implant assoc, cutaneous.

Question 13

Features of ALK+ AnaplasticLCL

Answer 13

• Younger, better prog
• t(2;5)
• Large cells, abundant cytoplasm
• Hallmark cells (eccentric nuclei/multiple nucleoli
• Doughnut cells

Question 14

Mutations in ALK- AnaplasticLCL

Answer 14

1. TP63
2. DUSP22 - JAK/STAT

Question 15

Treatment approaches in TLPDs