Anaplastic B-cell 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
Anaplastic B-cell lymphoma is one of 3 morphologic variants of diffuse large B cell lymphoma (DLBCL). The other 2 variants are immunoblastic lymphoma and centroblastic lymphoma.
Phenotype stem cell origin
The postulated normal counterpart is a germinal centre B cell or an activated post germinal centre B cell.
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 may test positive, unlike in other DLBCL. CD5 is positive in 10% of the cases. CD10 is expressed in approximately half of the cases.
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 may test positive, unlike in other DLBCL. CD5 is positive in 10% of the cases. CD10 is expressed in approximately half of the cases.
Epidemiology
It is a rare variant of DLBCL.
Clinics
The disease runs an aggressive course, as all DLBCL.
Pathology
The lymph node section shows large round or oval cells with pleomorphic nuclei resembling Hodgkins cells or anaplastic cells. It is morphologically indistinguishable from anaplastic large cell lymphoma of T-cell type. A sinusoidal and cohesive growth pattern may be observed.
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
Bibliography
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Citation
Gian Matteo Rigolin ; Francesco Cavazzini ; Antonio Cuneo
Anaplastic B-cell lymphoma
Atlas Genet Cytogenet Oncol Haematol. 2010-07-01
Online version: http://atlasgeneticsoncology.org/haematological/2093/anaplastic-b-cell-lymphoma
