Mantle cell lymphoma

Question 1

In which age group does mantle cell lymphoma occur?

Answer 1

Middle age and elderly

Question 2

What are the morphological features of mantle cell lymphoma?

Answer 2

Three growth patterns: mantle zone, nodular, diffuse

Mantle zone pattern - prominent mantle zone around normal/arophic germinal centres

Hyalinized blood vessels

Monotonous small lymphocytes with irregular, indented nuclei

Epithelioid histiocytes (can look starry sky)

Some cell may have larger nuclei with dispersed chromatin and higher prolif rate - blastoid variant

Question 3

What is the immunoprofile of MCL?

Answer 3

Pre-germinal centre immunophenotype

B-cell antigens +ve

CD5+ve (as opposed to FCL and MZL)

Ig +ve (usu IgM and IgD)

**Cyclin D1 +ve (nuclear)**

CD23 -ve (as opposed to SLL)

Usu -ve for CD10 and BCL-6

Question 4

What is the characteristic gene translocation in MCL?

Answer 4

t(11;14)(q13;32)

The CCND1 (cyclin D1) gene fuses with the IGH gene causing overexpression of Cyclin D1

This translocation only occurs in mantle cell lymphomas (apart from some plasma cell myelomas) so is very useful diagnostically

Question 5

What feature is useful in distinguishing MCL from FCL?

Answer 5

Absence of centroblasts and immunoblast-like cells

Question 6

What is the differential diagnosis of blastoid MCL?

Answer 6

T or B cell lymphoblastic lymphoma

Question 7

What is the median survival of MCL?

Answer 7

3-5 years