Name

Mantle cell lymphoma

ICD-O-1 Morphology

9621/3: Malignant lymphoma, lymphocytic, intermediate differentiation, NOS
9622/3: Malignant lymphoma, centrocytic

ICD-O-2 Morphology

9673/3: Mantle cell lymphoma
9674/3: Malignant lymphoma, centrocytic
Effective 1992 - 2000

ICD-O-3 Morphology

9673/3: Mantle cell lymphoma
Effective 2001 and later

Reportable

for cases diagnosed 1992 and later

Primary Site(s)

See Module 7
Most common sites of involvement: lymph nodes, gastrointestinal tract, and Waldeyer ring

Grade

Not Applicable

Module Rule

None

Alternate Names

Leukemic non-nodal mantle cell lymphoma
Malignant lymphoma, centrocytic [OBS]
Mantle cell lymphoma, blastic
Mantle cell lymphoma, CLL/SLL type
Mantle cell lymphoma, marginal zone-like
Mantle cell lymphoma, small cell
Mantle zone lymphoma [OBS]
MCL

Definition

Mantle cell lymphoma is a mature B-cell neoplasm usually composed of monomorphic small to medium-sized lymphoid cells with irregular nuclear contours.

Leukemic non-nodal mantle cell lymphoma is defined as mantle cell lymphoma in which the patient presents with peripheral blood, bone marrow, and sometimes splenic involvement but without significant adenopathy.

There are several variants of Mantle cell lymphoma

1) Blastoid: An aggressive variant where cells resemble lymphoblasts with dispersed chromatin and a high mitotic rate.

2) Pleomorphic: An aggressive variant where cells are pleomorphic, but many are large with oval to irregular nuclear contours, generally pale cytoplasm, and other prominent nucleoli in at least some of the cells.

3) Small-cell: Cells are small round lymphocytes with more clumped chromatin, either admixed or predominant, mimicking a small lymphocytic lymphoma

4) Marginal zone-like: There are prominent foci of cells with abundant pale cytoplasm resembling marginal zone or monocytoid B cells, mimicking a marginal zone lymphoma, sometimes these paler foci also resemble proliferation centers of chronic lymphocytic leukemia/small lymphocytic lymphoma

Abstractor Notes

Peripheral blood involvement common, present in almost all patients by flow cytometry. B symptoms in about half of patients.

Most patients present in Stage III or IV. Spleen, bone marrow and liver are common metastatic sites.

Patients presenting with peripheral blood, bone marrow and possibly splenic involvement but without adenopathy are reported to have better prognosis.

Definitive Diagnostic Methods

Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

Heavy and light chain genes rearranged, t(11;14)(q13;q32) in virtually all cases, overexpression CYCLIN D mRNA, mutation or deletions of ATM gene

Immunophenotyping

BCL2 positive
BCL6 negative
CD5 positive
CD10 negative
CD23 negative or weakly positive
CD43 positive
Cyclin D1 expression
FMC7 positive
IgM/IgD expression
IRF4 positive
MUM1 positive

Treatments

Chemotherapy
Immunotherapy
Radiation therapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Corresponding ICD-9 Codes

200.4 Mantle cell lymphoma

Corresponding ICD-10 Codes

C83.1 Diffuse non-Hodgkin's lymphoma, Small cleaved cell (diffuse)

Corresponding ICD-10-CM Codes (U.S. only)

C83.1 Mantle cell lymphoma (effective October 01, 2015)

Signs and Symptoms

Drenching night sweats
Fever (for no known reason)
Lymphocytosis
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph nodes
Skin rash or itchy skin
Weight loss (for no known reason)

Diagnostic Exams

CT (CAT) scan
Complete blood count (CBC)
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan

Progression and Transformation

None

Epidemiology and Mortality

Age: 60 years median age
Sex: male predominance

Sources

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature B-cell neoplasms
Pages: 285-290

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Glossary