Mantle cell lymphoma

Question 1

Tx goals

Answer 1

Usually not curative

Mainly to alleviate Sx

Question 2

OS with Tx

Answer 2

8-10 years

Question 3

Subset with indolent disease

Answer 3

Clinically: lymphocytosis, a few enlarged lymph nodes, and splenomegaly
SOX11 negativity
Ki-67 <30%

Question 4

Evaluation

Answer 4

HBV, HIV
BMB if cytopenias
LP if blastic variant
PETCT
Endoscopies if Sx
Echo
Fertility preservation

Question 5

Tx of young fit

Answer 5

age < 60, ECOG <2

BR / R-CHOP / Hyper- CVAD
followed by autologous HSCT
followed by R maintenance

Question 6

Tx of pts > 60

Answer 6

If fit and HSCT candidates- same as younger

If unfit BR and R maintenance

Question 7

R-Hyper-CVAD

Answer 7

Addition of cytarabine and methotrexate

Two courses A with CVAD B with only Cytarabine and Methotrexate

Better PFS but unacceptable toxicity

Question 8

R-DHAP

Answer 8

R
Dexamethasone
High dose Ara-C (cytarabine)
Platinol (Cisplatin)

Usually alternating R-CHOP/R-DHAP

Question 9

M:F

Answer 9

3:1

Question 10

Median age

Answer 10

68

Question 11

Types

Answer 11

Classic- nodal, Extranodal (GI)- SOX11 positive

Leukemic- Peripheral blood, BM, spleen, spares lymph nodes (more indolent)- SOX11 negative

Question 12

Advanced disease %

Answer 12

70-80%

Question 13

B symptoms %

Answer 13

33%

Question 14

Immunophnotype

Answer 14

Suraface IgM IgD
CD19, CD20, CD5 FMC7
SOX11
Cyclin D1
in Cyclin D1 negative- Cyclin D2/3 will be positive
Cyclin E- aggressive
P53 (variable)
CD23 negative

Question 15

Genetic featuresleo

Answer 15

t(11:14)- CCND1/IgH
Trosomy 12- 25%
TP53

Question 16

Extranodal disease

Answer 16

25%

mainly GI

Question 17

Waldeyer ring

Answer 17

שקדים, אנאואידים וקשריות תת-לשוניות

Question 18

Cytologic subtypes

Answer 18

Classic
Blastoid- aggressive
Pleomorphic- aggressive

Question 19

Bulky disease

Answer 19

25%

Question 20

high LDH

Answer 20

< 50%

Question 21

DD

Answer 21

Mainly CLL

Question 22

CLL vs MCL

Answer 22

CD5+ both
CD20- bright, CD23 negative, CD200 negative- MCL
CD20-dim, CD23+, CD200+- CLL
LEF1+- CLL
t(11,14)- MCL

Question 23

Lymphomatous polyposis

Answer 23

Rare presentation
Extensive polyps in GI tract
Anywhere along the tract
Most common distal ileum and colon

Question 24

MIPI

Answer 24

Age- 50, 60, 70
ECOG
LDH- 0.67, 1, 1.5
WBC- 6.7K, 10K 15K

Question 25

Tx of non autologous HSCT cadidtates

Answer 25

BR +/- addition of ibrutinib