Mantle cell lymphoma

Name
Mantle cell lymphoma
ICD-O-2 Morphology
9673/3
Effective 1992 - 2000
ICD-O-3 Morphology
9673/3
Effective 2001 and later
Reportable
for cases diagnosed 1992 and later
Primary Site(s)
See Abstractor Notes and Module 7

Help me code for diagnosis year :

Grade
6 - B-cell
Module Rule
None
Alternate Names
Malignant lymphoma, centrocytic [OBS]
Malignant lymphoma, lymphocytic, intermediate differentiation, diffuse [OBS]
Malignant lymphomatous polyposis
Mantle cell lymphoma
Mantle cell lymphoma, blastic
Mantle cell lymphoma, pleomorphic
Mantle cell lymphoma, small cell
Mantle zone lymphoma [OBS]
MCL
Definition
Lymphoma derived from subset of naive pre-germinal center cells, localized in primary follicles or in mantle region of secondary follicles; majority of cases displaying unmutated immunoglobulin heavy chain locus. Characterized by atypical small to medium-sized cells in nodular, diffuse, or mantle zone growth pattern.

An agressive type of B-cell non-Hodgkin lymphoma that usually occurs in middle-aged or older adults. It is marked by small to medium sized cancer cells that may be in the lymph nodes, spleen, bone marrow, and GI system.

Mantle cell lymphoma is a B-cell neoplasm generally composed of monomorphic small to medium-sized lymphoid cells with irregular nuclear contours and a CCND1 translocation.
Abstractor Notes
Typical patients are men. At diagnosis, the typical patient is in their 60s and usually presents with advanced disease. About half will have some combination of B symptoms. Swelling of lymph nodes and spleen are usually present. Bone marrow, liver and GI tract involvement occurs in a very high percentage.
Definitive Diagnostic Methods
Histological confirmation
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
CD5+
CD19+
CD20+
CD22+
CD23-
CD43+
CD79a+
Leu8+
Surface IgM and IgD; IgG in 20% of cases
Treatments
Chemotherapy
Immunotherapy
Radiation
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 (effective October 1, 2015 U.S. only)
C83.1 Mantle cell lymphoma
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None