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

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, blastic
Mantle cell lymphoma, CLL/SLL type
Mantle cell lymphoma, marginal zone-like
Mantle cell lymphoma, pleomorphic
Mantle cell lymphoma, small cell
Mantle zone lymphoma [OBS]
MCL
Definition
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.

Lymphomas has subset of naive pre-germinal center cells, localized in primary follicles or in mantle region of secondary follicles. Majority of cases display unmutated immunoglobulin heavy chain locus.

Morphologic variants of mantle cell lymphoma:
1. Blastoid
2. Pleomorphic
3. Small cell
4. Marginal zone-like
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
BCL6-
CD5+
CD10-
CD23- (or weakly positive)
CD43+
FMC-7+
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
Drenching night sweats
Fatigue
Fever (for no known reason)
Hepatosplenomegaly
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
Bone marrow aspiration and biopsy
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
Incidence: 3-10% of non-Hodgkin lymphomas
Sex: male predominance
Survival: 3-5 years median survival