Histiocyte-rich B-cell lymphoma
2010-07-01 Antonio Cuneo , Gian Matteo Rigolin , Francesco Cavazzini Affiliation1.Hematology Section, Department of Biomedical Sciences, University of Ferrara, Corso Giovecca 203, Ferrara, Italy
Clinics and Pathology
Disease
T-cell/histiocyte-rich large B cell lymphoma (THRLBCL) is a distinct entity of aggressive lymphoma, recognized by the WHO classification as a separate entity (2008), in which few scattered neoplastic cells (usually
Phenotype stem cell origin
The postulated normal counterpart is a germinal centre B cell.
The immunophenotype of the neoplastic component in pan-B positive, BCL6+, CD15-, CD30-.
The immunophenotype of the neoplastic component in pan-B positive, BCL6+, CD15-, CD30-.
Epidemiology
It accounts for a minority of diffuse large B-cell lymphoma (
Clinics
The disease runs an aggressive course and is usually associated with poor outcome in those patients presenting at an advanced stage.
Pathology
The lymph node section shows scattered large cells surrounded by many lymphocytes and histiocytes. The disease must be distinguished from nodular lymphocyte-predominant Hodgkins lymphoma, which has distinct clinical features.
Treatment
Chemoimmunotherapy using anti CD20 monoclonal antibody rituximab in combination with CHOP or CHOP-like regimens is the standard of care (El Weshi et al., 2007).
Prognosis
A >80% overall response rate was obtained by chemoimmunotherapy, with a 5-year overall survival of approximately 50% (El Weshi et al., 2007).
Genes Involved and Proteins
Article Bibliography
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Citation
Antonio Cuneo ; Gian Matteo Rigolin ; Francesco Cavazzini
Histiocyte-rich B-cell lymphoma
Atlas Genet Cytogenet Oncol Haematol. 2010-07-01
Online version: http://atlasgeneticsoncology.org/haematological/2071/histiocyte-rich-b-cell-lymphoma
