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 <5%) are surrounded by reactive T-lymphocytes and histiocytes. THRLBCL shares several morphological and immunophenotypic similarities with nodular lymphocyte-predominant Hodgkins lymphoma (Pittaluga et al., 2010). These two entities may share initial transforming events that occur at germinal center B cell, followed by early divergence in the evolution of the neoplastic process (Franke et al., 2002).

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 (<10%).

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
177867122007T-cell/histiocyte-rich B-cell lymphoma: Clinical presentation, management and prognostic factors: report on 61 patients and review of literature.El Weshi A et al
124145322002Comparative genomic hybridization pattern distinguishes T-cell/histiocyte-rich B-cell lymphoma from nodular lymphocyte predominance Hodgkin's lymphoma.Franke S et al
8683394199614q+ chromosome marker in a T-cell-rich B-cell lymphoma.La Starza R et al
202078402010T-cell/histiocyte-rich large B-cell lymphoma.Pittaluga S et al
159429422005PAX5/IGH rearrangement is a recurrent finding in a subset of aggressive B-NHL with complex chromosomal rearrangements.Poppe B et al
158949212005T-cell/histiocyte-rich large B-cell lymphoma displays a heterogeneity similar to diffuse large B-cell lymphoma: a clinicopathologic, immunohistochemical, and molecular study of 30 cases.Wang J et al
168569722006T-cell/histiocyte-rich large B-cell lymphoma associated with a near-tetraploid karyotype and complex genetic abnormalities.de Leval L et al

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

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