T Cell Lymphomas

Question 1

ALK+ Anaplastic large cell lymphoma has what prognosis?

Answer 1

Good

Question 2

Anaplastic large cell lymphoma in general has a ____ prognosis compared to PTCL?

Answer 2

Much better

Question 3

GATA-3 denotes what prognosis in PTCL?

Answer 3

Poor

Question 4

Initial amnagement of PTCL

Answer 4

CHO(E)P

Question 5

Initial management of PTCL that is CD30+

Answer 5

BV-CHP

Question 6

Who should not get etoposide added to inital management in PTCL?

Answer 6

Age >60

Question 7

When should we take PTCL to autoSCT?

Answer 7

First CR

Question 8

Treatment for localized nasal NK/T cell lymphoma in an unfit patient?

Answer 8

RT alone

Question 9

Treatment for localized nasal NK/T cell lymphoma in a fit patient?

Answer 9

Sequential or concurrent chemo then RT
SMILE then RT
LVP then RT

VIC+RT
Cis+RT then VIPD

Question 10

Treatment for disseminated NK/T-cell lymphoma? (2)

Answer 10

SMILE +/- RT
DDGP (Cisplatin, Dex, Gem, Asp)

Question 11

Treatment for breast implant assocaited anaplastic LCL?

Answer 11

Remove the implant

Question 12

MoA of Mogamulizumab?

Answer 12

Anti-CCR4 mAB

Question 13

1L Management of early stage CTCL? (4)

Answer 13

Steroids
Bexarotene
PUVA
Localized RT

Question 14

2L treatment of early stage CTCL? (3)

Answer 14

Total skin electron
Interferon-alpha
MTX

Question 15

1L treatment for advanced CTCL?

Answer 15

Steroids, PUVA, RT
MTX
ECP
Gem
Doxil

Question 16

Preferred front line regimen for ALK+ ALCL?

Answer 16

BV-CHP

Question 17

Treatment of unicentric Castleman’s diseae?

Answer 17

Surgical resection of the involved LN

Question 18

What is the definition of unicentric vs multicentric Castleman’s disease?

Answer 18

Unicentric only involves a single enlarged LN.
Multicentric involves multiple, usually has severe symptoms and HHV-8 infection

Question 19

What infection is associated with multicentric Castleman’s disease?

Answer 19

HHV-8

Question 20

Treatment of active Multicentric Castleman’s disease with organ failure?

Answer 20

R-CHOP
R monotherapy

Question 21

Treatment for multicentric Castleman’s disease without organ failure in patients who are HIV negative and HHV8 negative?

Answer 21

Siltuximab

Question 22

Treatment for multicentric Castleman’s disease without organ failure in patients who are HIV+ and/or HHV-8+?

Answer 22

Rituximab

Question 23

3 criteria for active Castlemans disease?

Answer 23

Fever
Increased CRP >20
At least 3 MCD symptoms: LAD, splenomegaly, edema, effusion, ascites, cough, nasal obstruction, xerostomia, rash, neuro sx, jaundice, AIHA

Question 24

Immunophenotype of Adult T-cell leukemia/lymphoma?

Answer 24

CD2+, CD3+. CD4+, CD5+,
CD7-, CD8-
CD25++, CD29+
Negative for CD10, 19, 20)

Question 25

What is Erdheim Chester disease?

Answer 25

Multisystem histiocyte disorder that leads to infiltration of organ systems. Usually has osteosclerotic lesions in long bones.

Question 26

Treatment of Erdheim Chester disease with BRAF V600E mutation?

Answer 26

vemurafenib

Question 27

Patient presents with a pleural effusion, ascites, generalized lymphadenopathy, fever, and hepatosplenomegaly. LN biopsy shows a CD3+, CD4+, CD7+, CD8+ lymphoma. Diagnosis?

Answer 27

Aitl

Question 28

Preferred treatment for AITL (4)

Answer 28

Bv-CHP (for CD30+) CHOEP CHOP DA-EPOCH

Question 29

Treatment for primary cutaneous ALCL that is localized

Answer 29

Surgery +/- ISRT or ISRT alone

Question 30

Treatment for primary cutenaous ALCL taht is multifocal but no visceral disease

Answer 30

Bv

Question 31

Treatment of ERdheim chester disease with a MAPK mutation?

Answer 31

Cobimetinib

Question 32

Treatment of Erdheim Chester disease without any special mutation?

Answer 32

Cladribine