Mantle cell lymphoma

Question 1

Translocation to know for MCL

Answer 1

t(11;14)

Question 2

MCL workup

Answer 2

PET/CT
- Given neurologic symptoms AND once WBC count declining (don’t seed CSF) OR blastoid variant (higher CNS risk), plan for LP with cytology and flow cytometry of CSF (Some concern about seeding re CSF)

Question 3

Immunophenotype

Answer 3

CD5+, CD23-, CD10-, SOX11+, Cyclin D1

Question 4

Management of limited stage

Answer 4

• Given asymptomatic AND low risk disease AND no indication for treatment, initial observation period until symptomatic
• IF indicated for treatment, BR
  *involved field radiation if can be encompassed in single radiation field

Question 5

What is TRIANGLE?

Answer 5

R-CHOP/R-DHAP + ibrutinib without transplant

Question 6

Maintenance therapy for MCL?

Answer 6

IF post auto-HSCT, rituxan maintenance (Phase III LYSA – PFS< OS benefit for patients <66 most people do, but not clear consensus given MAINTAIN below)
***For pts w/ BR induction, no maintenance rituxan (MAINTAIN – no PFS or OS benefit)

Question 7

Relapsed refractory MCL management

Answer 7

aggressive disease - CAR-T
nonaggressive - IF no indication for treatment, initial period of observation
IF older or frail → BTK – acalabrutinib vs. zanubritinib vs. pirtobrutinib (Preferred – but outcomes quite poor)

Question 8

Third line for MCL

Answer 8

Brexicel (high response rates and deep remissions)
*pirtobrutinib is also an option if ineligible for CAR-T

Question 9

Management of limited stage MCL

Answer 9

XRT