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Diffuse large cell lymphoma

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

(Note : for Links provided by Atlas : click)

Identity

ICD-Topo C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
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

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

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.

Bibliography

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PMID 10907484
 
Deregulation of BCL6 in non-Hodgkin lymphoma by insertion of IGH sequences in complex translocations involving band 3q27.
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Acquired chromosome 11q deletion involving the ataxia teleangiectasia locus in B-cell non-Hodgkin's lymphoma: correlation with clinicobiologic features.
Cuneo A, Bigoni R, Rigolin GM, Roberti MG, Milani R, Bardi A, Minotto C, Agostini P, De Angeli C, Narducci MG, Sabbioni S, Russo G, Negrini M, Castoldi G
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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.org/Anomalies/DLCLID2076.html


Other genes implicated (Data extracted from papers in the Atlas) [ 61 ]

Genes ADAM10 AKT1 ALK BACH2 BCL10 CCND1 BCL2L1 BCL6 NBEAP1 BCLAF1
BIRC2 CASP8AP2 CASP9 CCNB1 CCR1 CD38 CPM DNMT3B E2F1 E2F3
EGR1 EIF4B EML4 EZH2 FAS FBXO11 FCGR2B FMNL1 FOXP1 GLI2
HTRA2 ID4 IGH IGH IGK IGL IL21R IRF4 MAPK4
MIR200C MKI67 MME NAMPT NME1 P2RY8 TP53 PASD1 PIK3CD PRDM1
PRDM2 PTPRA LMO2 RUVBL1 SYK TET2 TNFAIP3 TP53BP2 YBX1 ZAP70
ZBTB7A

External links

COSMICHisto = - Site = haematopoietic_and_lymphoid_tissue (COSMIC)
arrayMapTopo ( C42) Morph ( 9680/3) - arrayMap (UZH-SIB Zurich)  [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]
Disease databaseDiffuse large cell lymphoma
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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