Centroblastic lymphoma
2010-07-01 Gian Matteo Rigolin  , Francesco Cavazzini  , Antonio Cuneo   Affiliation1.Hematology Section, Department of Biomedical Sciences, University of Ferrara, Corso Giovecca 203, Ferrara, Italy
Clinics and Pathology
Disease
Centroblastic lymphoma is one of 3 morphologic variants of diffuse large B cell lymphoma (DLBCL). The other 2 variants are Immunoblastic lymphoma and anaplastic B-cell lymphoma.
The disease is not recognized as a separate entity in the WHO classification (2008).
The disease is not recognized as a separate entity in the WHO classification (2008).
Phenotype stem cell origin
The postulated normal counterpart is a germinal centre B cell or an activated post germinal centre B cell.
The immunophenotype may help distinguishing germinal centre-derived DLBCL from activated B-cell derived DLBCL, the former being CD10+ (>30% of the cells), or BCL6+ and IRF4/MUM1-.
The immunophenotype reproduces that of DLBCL, with pan B-cell markers positive, surface and/or cytoplasmic Ig positive in the majority of the cases. The CD30 antigen is negative. CD5 is positive in 10% of the cases. CD10 is expressed in approximately half of the cases.
The immunophenotype may help distinguishing germinal centre-derived DLBCL from activated B-cell derived DLBCL, the former being CD10+ (>30% of the cells), or BCL6+ and IRF4/MUM1-.
The immunophenotype reproduces that of DLBCL, with pan B-cell markers positive, surface and/or cytoplasmic Ig positive in the majority of the cases. The CD30 antigen is negative. CD5 is positive in 10% of the cases. CD10 is expressed in approximately half of the cases.
Epidemiology
It is the most common morphologic variant of DLBCL.
Clinics
The disease runs an aggressive course, as all DLBCL.
Pathology
The lymph node section shows an overwhelming infiltrate by medium-to-large size cells with vescicular nuclei and membrane bound nucleoli. The cytoplasm is scanty. In the majority of cases centroblasts are admixed with immunoblasts (
Treatment
Chemoimmunotherapy using anti CD20 monoclonal antibody rituximab in combination with CHOP or CHOP-like regimens is the standard of care.
Prognosis
Chemoimmunotherapy may cure 40-60% of the cases depending on age and risk factors.
Genes Involved and Proteins
Article Bibliography
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Citation
Gian Matteo Rigolin ; Francesco Cavazzini ; Antonio Cuneo
Centroblastic lymphoma
Atlas Genet Cytogenet Oncol Haematol. 2010-07-01
Online version: http://atlasgeneticsoncology.org/haematological/2091/centroblastic-lymphoma
