Mantle Cell Lymphoma

Question 1

What is the median age of presentation of mantle cell lymphoma?

Answer 1

60-65y

Question 2

What is the median survival?

Answer 2

4-5y (8-12y in young fit pts who tolerate intensive tx)

Question 3

What percentage present with advanced disease?

Answer 3

> 90%

Question 4

What histological subtype confers a worse prognosis?

Answer 4

Blastoid

Question 5

What percentage have CNS relapse and when does this tend to occur?

Answer 5

5-8%

At 15-20months

Question 6

What 4 factors are used in the MIPI prognostication tool?

Answer 6

Age, PS, LDH, WCC

Question 7

What management would you suggest for early (stage 1A and 2A) disease?

Answer 7

Involved field irradiation

Question 8

What percentage have microscopic GI disease?

Answer 8

> 90%

Question 9

What 2nd line agent is offered that can cross the BBB?

Answer 9

Ibrutinib (BTK inhibitor)

Question 10

What would be your management plan for a young fit patient with advanced disease?

Answer 10

High dose cytarabine containing regimen with rituximab followed by BEAM autoSCT if CR/PR, followed by maintenance rituximab for 3y.

4y PFS 83%

Question 11

What would your management plan be for a patient who is symptomatic with advanced disease but unfit for ASCT?

Answer 11

R-CHOP (R-bendamustine of elderly) followed by maintenance rituximab if CR/PR