99 Mantle Cell Lymphoma

Question 1

Other names for mantle cell lymphoma

Answer 1

Centrocytic lymphoma
Intermediate lymphocytic lymphoma

Question 2

Morphologic description of MCL

Answer 2

Small- to intermediate-size cells with irregular, cleaved nuclei; dense chromatin; and indistinct nucleoli

Question 3

TRUE OR FALSE

A leukemic non-nodal variant, resembling chronic lymphocytic leukemia (CLL), associated with missing SOX-11 expression, may be associated with a more indolent course.

Answer 3

TRUE

A leukemic non-nodal variant, resembling chronic lymphocytic leukemia (CLL), associated with missing SOX-11 expression, may be associated with a more indolent course.

In contrast, the blastoid cell variant, including a blastic and a pleomorphic phenotype, displays a more aggressive clinical course.

Question 4

The immunophenotype of the cells in MCL

Answer 4

Coexpression of B-cell antigens (CD19+, CD20+, CD22+, CD43+, CD79+, secretory immunoglobulin [sIg] M+, sIgD+), and the T-cell–associated marker CD5+

Question 5

MCL cells stain strongly for the antiapoptotic protein BCL-2 but are negative for germinal center markers such as______________

Answer 5

CD10 and BCL-6

Question 6

The median age at presentation is approximately

Answer 6

65 years

Twice as high in males and increases with age
No specific etiologic agent has been associated with MCL.

Question 7

Chromosomal translocation in MCL; the primary event in the pathogenesis of MCL

Answer 7

t(11;14)

A low copy number of the t(11;14) translocation has been found in the blood cells of 1% to 2% of healthy individuals without evidence of clinical disease

Question 8

The chromosomal translocation t(11;14) results in overexpression of

Answer 8

cyclin D1

The very rare cyclin D1–negative cases usually overexpress cyclin D2 or D3

Question 9

TRUE OR FALSE

Cell-cycle dysregulation is a hallmark of MCL.

Answer 9

TRUE

Cell-cycle dysregulation is a hallmark of MCL.

Question 10

TRUE OR FALSE

SOX11 expression leads to progression to classical MCL.

Answer 10

TRUE

SOX11 expression leads to progression to classical MCL.

Question 11

A cell cycle–related protein, as determined by immunohistochemistry, has been prospectively confirmed as a reliable prognostic marker that allows the identification of high-risk patients

Answer 11

Ki-67 expression

High-risk patients (Ki-67 >30%)
Independent of clinical features and adds prognostic weight to the Mantle Cell Lymphoma International Prognostic Index (MIPI) score

Question 12

Oncogenic alterations that result in “transformation to more aggressive blastoid variants”

Answer 12

TP53 mutations

Question 13

In 25% of cases, there is symptomatic gastrointestinal (GI) involvement, typically presenting as

Answer 13

Polyposis coli

Marrow is involved with MCL in the vast majority of patients, and 50% of patients present with blood involvement, sometimes with an overt leukemic phase.

Question 14

Prognostic parameters in MCL include

Answer 14

Serum level of β2-microglobulin and LDH, blastoid cytology, age, Ann Arbor stage, extranodal presentation, and constitutional symptoms

Question 15

The Mantle Cell Lymphoma International Prognostic Index (MIPI) score’s four independent prognostic factors:

Answer 15

Age, performance status, LDH, and leukocyte count

Question 16

Difference of immunophenotype between MCL and CLL

Answer 16

CD23 is typically highly expressed in CLL

Question 17

Difference of immunophenotype between MCL and FL

Answer 17

Like FL, MCL is positive for CD20 and BCL-2, but in contrast to follicular lymphoma, MCL is negative for CD10 and BCL-6

Question 18

Therapy for localized stage disease

In general, MCL is still considered incurable, and most patients follow an aggressive clinical course.

Localized disease with low tumor burden is rare.

Answer 18

Shortened chemotherapy induction followed by radiation consolidation

Question 19

Offered to patients with advanced stage who are medically unfit patients and who are not able to tolerate cytotoxic therapy

Answer 19

Rituximab monotherapy

Question 20

The standard of care in both first-line and relapsed settings for patients with advanced-stage MCL

Answer 20

Immunochemotherapy

Complete remission rates are only 30% to 40% with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP), typically with a short duration of response of 10–12 months.

Question 21

Represents the currently most frequently applied approach in older patients, based on its favorable tolerability

Answer 21

Bendamustine–rituximab (BR)

BR may be preferable, especially in patients with CLL-like presentation, whereas CHOP-like regimens seems to be appropriate in the more aggressive cases with elevated LDH.

Question 22

Chemotherapy of choice for blastoid variants

Answer 22

Cytarabine-containing regimens

Question 23

Regimen that has been proven to result in superior long-term outcome compared with R-CHOP

Answer 23

Bortezomib-containing VR-CAP

Question 24

First line treatment for young patient (< 65 years of age)

Answer 24

Dose-intensified immunochemotherapy (RCHOP, High dose Ara-C)

–Autologous SCT
–Rituximab maintenence

Question 25

First line treatment for elderly patient (older than 65 years of age)

Answer 25

Conventional immunochemptherapy (VR-CAP, RCHOP, BR, R-BAC)

–Rituximab maintenence

Question 26

First line treatment for compromised patient

Answer 26

Best supportive care
R-chlorambucil
BR (dose-reduced)
R-CVP