Diffuse large cell lymphoma

2000-05-01   Gian Luigi Castoldi , Antonio Cuneo 

1.Hematology Section, Dept. Of Biomedical Sciences, University of Ferrara, 44100 Ferrara Italy

Clinics and Pathology

Disease

diffuse large cell lymphoma (DLCL) includes centroblastic lymphoma, B-cell immunoblastic lymphoma and B-cell large cell anaplastic lymphoma in the Kiel classification; this neoplasia may present as a de novo condition or it may derive from the transformation of follicle centre cell lymphoma or, less frequently, of marginal zone lymphoma

Phenotype stem cell origin

the tumor cells are pan-B+ and CD45+; CD5 and CD10 are positive in a minority of cases; positivity for surface Ig is found in the majority of cases, a minority of which also show intracytoplasmic Ig; some cases of the anaplastic subtype may be CD30+; usually, BCL6 is positive in cases with predominant centroblastic morphology, whereas syndecan-1 (CD138) tests positive in the immunoblastic variant; the putative normal cellular counterparts are cells of follicle / post follicle centre origin that have encountered the antigen and harbour somatic hypermutations of the Ig-gene variable region

Pathology

the cells are large, with vesicular nuclei at least twice the size of the nucleus of a small lymphocyte; there is a mixture of cells resembling centroblasts and immunoblasts; in rarer cases the cells are morphologically indistinguishable from those seen in anaplastic lymphoma of T- or null cell type

Note

the large majority of cases show clonal cytogenetic lesions; some of these changes are associated with a known primary genetic defect responsible for lymphomagenesis, whereas a plethora of additional changes may be involved in tumor progression.
most studies failed to establish the prognostic predictivity for any primary chromosome defect, whereas there is evidence that several secondary aberrations may affect prognosis.

Bibliography

Pubmed IDLast YearTitleAuthors
109074841999Interphase FISH in chronic lymphoproliferative disorders and comparative genomic hybridisation in the study of lymphomas.Bentz M et al
98242061998Deregulation of BCL6 in non-Hodgkin lymphoma by insertion of IGH sequences in complex translocations involving band 3q27.Chaganti SR et al
108932932000Acquired chromosome 11q deletion involving the ataxia teleangiectasia locus in B-cell non-Hodgkin's lymphoma: correlation with clinicobiologic features.Cuneo A et al
105778571999World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues: report of the Clinical Advisory Committee meeting-Airlie House, Virginia, November 1997.Harris NL et al
82082681994Rearrangement of the bcl-6 gene as a prognostic marker in diffuse large-cell lymphoma.Offit K et al
20093701991Cytogenetic analysis of 434 consecutively ascertained specimens of non-Hodgkin's lymphoma: clinical correlations.Offit K et al
105561971999Clinicopathogenetic significance of chromosomal abnormalities in patients with blastic peripheral B-cell lymphoma. Kiel-Wien-Lymphoma Study Group.Schlegelberger B et al
94466601998Application of interphase fluorescence in situ Hybridization for the detection of the Burkitt translocation t(8;14)(q24;q32) in B-cell lymphomas.Siebert R et al
75207751994Stromal cell lines derived from LP-BM5 murine leukemia virus-infected long-term bone marrow cultures impair hematopoiesis in vitro.Tse KF et al

Citation

Gian Luigi Castoldi ; Antonio Cuneo

Diffuse large cell lymphoma

Atlas Genet Cytogenet Oncol Haematol. 2000-05-01

Online version: http://atlasgeneticsoncology.org/haematological/2076/diffuse-large-cell-lymphoma

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