Histiocyte-rich B-cell lymphoma

2010-07-01   Antonio Cuneo , Gian Matteo Rigolin , Francesco Cavazzini 

1.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-.

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).

Bibliography

Pubmed IDLast YearTitleAuthors

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/case-report-explorer/img/js/template.js