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Lymphoepithelioid lymphoma

Written2002-06Antonio Cuneo, Gianluigi Castoldi
Hematology Section, Department of Biomedical Sciences, University of Ferrara, Corso Giovecca 203, Ferrara, Italy

(Note : for Links provided by Atlas : click)

Identity

ICD-Topo C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
Atlas_Id 2005
Other namesLennert's lymphoma

Clinics and Pathology

Phenotype / cell stem origin Peripheral CD4+ T-cell lymphoma
Epidemiology The disease is rare
Clinics The patients present superficial lymph node involvement. The cervical areas are predominantly affected, whereas thoracic adenopathies and deep abdominal involvement occur unfrequently at presentation.
Pathology The disease cannot be separated from the broad category of peripheral T-cell lymphoma (PTL). PTL is characterized by a heterogeneous cellular composition with small and large cells with an inflammarory background. Lennert's lymphoma can be recognized by the presence of numerous epithelioid histiocytes usually grouped in small clusters. Lennert's lymphoma is not considered as a distinct clinicopathological entity.
Treatment The disease must be treated with multiagent chemotherapy and/or radiation therapy as for other PTL
Evolution The disease usually runs a relatively aggressive clinical course.
Prognosis Median survival of 16 to 32 months was reported in some studies

Cytogenetics

Note Clonal aberrations are reported in the vast majority of cases. Chromosome 3 is frequently involved: trisomy 3 ; 3q rearrangements (duplication of bands 3q22-24 or 3q22 breaks) are recurrent abnormalities ; A 6q- chromosome was also reported.

Bibliography

Other peripheral T-cell lymphomas.
Delmer A, Zittoun R
Magrath I (Ed) The non Hodgkin's lymphomas 2nd edition..
 
Cytogenetic and immunohistochemical analysis of lymphoepithelioid cell lymphoma (Lennert's lymphoma): further substantiation of its T-cell nature.
Gödde-Salz E, Feller AC, Lennert K
Leukemia research. 1986 ; 10 (3) : 313-323.
PMID 3081766
 
Cytogenetic studies in non-Hodgkin lymphomas--results from surgical biopsies.
Kristoffersson U, Heim S, Olsson H, Akerman M, Mitelman F
Hereditas. 1986 ; 104 (1) : 1-13.
PMID 3710822
 

Citation

This paper should be referenced as such :
Cuneo, A ; Castoldi, GL
Lymphoepithelioid lymphoma
Atlas Genet Cytogenet Oncol Haematol. 2002;6(4):290-290.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/LymphoepithLymphoID2005.html


Other genes implicated (Data extracted from papers in the Atlas) [ 2 ]

Genes IGK IGK

External links

arrayMap (UZH-SIB Zurich)
COSMICHisto = - Site = haematopoietic_and_lymphoid_tissue (COSMIC)
arrayMap[select an item]
 
 
Disease databaseLymphoepithelioid lymphoma
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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