Definition/General
Introduction:
Mantle cell lymphoma (MCL) is a distinct B-cell neoplasm characterized by cyclin D1 overexpression
It accounts for 3-10% of all non-Hodgkin lymphomas
MCL shows aggressive clinical behavior with poor overall survival
It is derived from naive B-cells in the mantle zone.
Origin:
Arises from mantle zone B-cells surrounding germinal centers
These cells are unmutated and express IgM/IgD
MCL cells retain naive B-cell phenotype
t(11;14)(q13;q32) translocation is characteristic
Results in CCND1-IGH fusion.
Classification:
Classified under mature B-cell neoplasms (WHO 2016)
Nodal type (classical)
Leukemic non-nodal type
In situ mantle cell neoplasia
Indolent MCL
Pleomorphic variant
Blastoid variant.
Epidemiology:
Median age 60-65 years
Male predominance (3-4:1)
Rare in children and young adults
Higher incidence in Western countries
Represents 6% of all lymphomas
Incidence increasing with improved recognition.
Clinical Features
Presentation:
Generalized lymphadenopathy (80-90%)
Splenomegaly (50-60%)
Hepatomegaly (25-30%)
Extranodal involvement (GI tract, bone marrow)
Waldeyer ring involvement
CNS involvement (rare).
Symptoms:
B-symptoms (fever, night sweats, weight loss)
Fatigue and weakness
Abdominal discomfort (splenomegaly)
GI symptoms (nausea, diarrhea)
Bleeding (thrombocytopenia)
Recurrent infections.
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Gross Description
Microscopic Description
Immunohistochemistry
Positive Markers:
CD20 (positive)
PAX5 (positive)
Cyclin D1 (positive - diagnostic)
SOX11 (positive)
CD79a (positive)
IgM (positive)
IgD (positive).
Negative Markers:
CD23 (negative - distinguishes from CLL)
CD10 (negative)
BCL6 (negative)
CD21 (negative)
CD35 (negative)
TdT (negative).
Diagnostic Utility:
Cyclin D1 is pathognomonic
SOX11 helps in cyclin D1-negative cases
CD23 negativity distinguishes from CLL/SLL
CD10/BCL6 negativity excludes follicular lymphoma.
Molecular/Genetic
Differential Diagnosis
Sample Pathology Report
Template Format
Sample Pathology Report
Complete Report: This is an example of how the final pathology report should be structured for this condition.
Specimen Information
[specimen type], [location], measuring [size] cm
Architecture
Shows [diffuse/nodular] pattern with [complete/partial] effacement
Morphology
[Small/medium] sized lymphoid cells with [nuclear features]
Morphological Variant
[Classical/pleomorphic/blastoid] variant
Immunohistochemistry
CD20: [+], Cyclin D1: [+], CD23: [-], SOX11: [+/-]
Proliferation Index
Ki-67: [X]% (proliferation index)
Molecular Studies
t(11;14): [positive/negative], CCND1 rearrangement: [present/absent]
Staging
Stage: [I/II/III/IV], bone marrow: [involved/uninvolved]
Prognostic Factors
MIPI score: [low/intermediate/high], Ki-67: [X]%
Final Diagnosis
Final diagnosis: Mantle cell lymphoma, [variant], Stage [X]