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Immunoblastic lymphoma

Written2010-07Antonio Cuneo, Gian Matteo Rigolin, Francesco Cavazzini
Hematology Section, Department of Biomedical Sciences, University of Ferrara, Corso Giovecca 203, Ferrara, Italy

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Identity

ICD-Topo C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
ICD-Morpho 9684/3
Atlas_Id 2092

Clinics and Pathology

Disease Immunoblastic lymphoma is one of 3 morphologic variants of diffuse large B cell lymphoma (DLBCL). The other 2 variants are centroblastic lymphoma and anaplastic B-cell lymphoma.
The disease is not recognized as a separate entity in the WHO classification (2008).
Phenotype / cell stem origin The postulated normal counterpart is a germinal centre B cell or an activated post germinal centre B cell.
The immunophenotype may help distinguishing germinal centre-derived DLBCL from activated B-cell derived DLBCL, the former being CD10+ (>30% of the cells), or BCL6+ and IRF4/MUM1-.
The immunophenotype reproduces that of DLBCL, with pan B-cell markers positive, surface and/or cytoplasmic Ig positive in the majority of the cases. The CD30 antigen is negative. CD5 is positive in 10% of the cases. CD10 is expressed in approximately half of the cases.
Epidemiology It accounts for approximately 1/4 of DLBCL.
Clinics The disease runs an aggressive course, as all DLBCL.
Pathology The lymph node section shows an overwhelming infiltrate (>90%) by medium-to-large size cells with centrally located nucleolus and fairly abundant basophilic cytoplasm.
Treatment Chemoimmunotherapy using anti CD20 monoclonal antibody rituximab in combination with CHOP or CHOP-like regimens is the standard of care.
Prognosis Chemoimmunotherapy may cure 40-60% of the cases depending on age and risk factors.

Cytogenetics

Cytogenetics Molecular Because the distinction from other morphologic variants of DLBCL is not reproducible it is generally accepted that there are not distinctive cytogenetic or molecular features with respect to diffuse large B-cell lymphoma.
However, some authors described a more frequent occurrence of losses of the whole chromosome 10, deletions in 8q and 14q, as well as structural abnormalities of 4q in immunoblastic lymphoma than in other morphologic variants of DLBCL (Schlegelberger et al., 1999).

Bibliography

Clinicopathogenetic significance of chromosomal abnormalities in patients with blastic peripheral B-cell lymphoma. Kiel-Wien-Lymphoma Study Group.
Schlegelberger B, Zwingers T, Harder L, Nowotny H, Siebert R, Vesely M, Bartels H, Sonnen R, Hopfinger G, Nader A, Ott G, Muller-Hermelink K, Feller A, Heinz R.
Blood. 1999 Nov 1;94(9):3114-20.
PMID 10556197
 
Diffuse large B-cell lymphoma, not otherwise specified.
Stein H, Warnke RA, Chan WC, Jaffe ES, Chan JKC, Gatter KC, Campo E.
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW, ed. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. WHO press, 4th ed. Lyon: IARC. 2008; pp 233-237.
 

Citation

This paper should be referenced as such :
Cuneo, A ; Rigolin, GM ; Cavazzini, F
Immunoblastic lymphoma
Atlas Genet Cytogenet Oncol Haematol. 2011;15(4):356-357.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/ImmunoLymphoID2092.html


Other genes implicated (Data extracted from papers in the Atlas) [ 1 ]

Genes NUDT6

External links

COSMICHisto = - Site = haematopoietic_and_lymphoid_tissue (COSMIC)
arrayMapTopo ( C42) Morph ( 9684/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
 
 
Disease databaseImmunoblastic lymphoma
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