Diffuse large cell lymphoma

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Written2000-05Antonio Cuneo, Gian Luigi Castoldi
Hematology Section, Dept. Of Biomedical Sciences, University of Ferrara, 44100 Ferrara Italy

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1. Identity

ICD-Morpho 9680/3 Diffuse large B-cell lymphoma (DLBCL), NOS; Primary DLBCL of the CNS; Primary cutaneous DLBCL, leg type; EBV positive DLBCL of the elderly; DLBCL associated with chronic inflammation; B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and Burkitt lymphoma
Atlas_Id 2076

2. 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 / cell stem 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

3. Genetics

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.

4. Bibliography

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Deregulation of BCL6 in non-Hodgkin lymphoma by insertion of IGH sequences in complex translocations involving band 3q27.
Chaganti SR, Rao PH, Chen W, Dyomin V, Jhanwar SC, Parsa NZ, Dalla-Favera R, Chaganti RS
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Acquired chromosome 11q deletion involving the ataxia teleangiectasia locus in B-cell non-Hodgkin's lymphoma: correlation with clinicobiologic features.
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Rearrangement of the bcl-6 gene as a prognostic marker in diffuse large-cell lymphoma.
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Application of interphase fluorescence in situ Hybridization for the detection of the Burkitt translocation t(8;14)(q24;q32) in B-cell lymphomas.
Siebert R, Matthiesen P, Harder S, Zhang Y, Borowski A, Zühlke-Jenisch R, Metzke S, Joos S, Weber-Matthiesen K, Grote W, Schlegelberger B
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Stromal cell lines derived from LP-BM5 murine leukemia virus-infected long-term bone marrow cultures impair hematopoiesis in vitro.
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5. Citation

This paper should be referenced as such :
Cuneo, A ; Castoldi, GL
Diffuse large cell lymphoma
Atlas Genet Cytogenet Oncol Haematol. 2000;4(2):83-84.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://atlasgeneticsoncology.usal.es/classic/Anomalies/DLCLID2076.html

6. External links

COSMICHisto = - Site = haematopoietic_and_lymphoid_tissue (COSMIC)
arrayMap (UZH-SIB Zurich)Topo ( C42) Morph ( 9680/3) -   [auto + random 100 samples .. if exist ]   [tabulated segments]
Other databaseTumor Portal - Broad Institute
Other databaseLymphoid Neoplasm Diffuse Large B-cell Lymphoma (DLBC) TCGA Copy Number Portal
Other databaseDiffuse B cell lymphoma ( intOGen )
Other databaseLymphoma Overview - Disease Synopsis [canSAR]
Other databaseLymphoid Neoplasm Diffuse Large B-cell Lymphoma [ Genomic Data Commons - NCI TCGA-DLBC]
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed

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