t(6;14)(p22;q32) IGH/ID4

2008-04-01   Jean-Loup Huret 

1.Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France

Clinics and Pathology

Disease

B-cell acute lymphoblastic leukaemia (B-ALL)

Phenotype stem cell origin

Pre-B ALL: CD10+ in 14 of 15 cases, CD19+ probably in the remaining case.

Epidemiology

15 cases available: 9 male and 6 female patients; mediane age was 16 years (range: 6 - 48 years).

Clinics

Complete remission was obtained in 15 of 15 cases.
Atlas Image

Prognosis

Prognosis looks fair (see survival curve).

Genes Involved and Proteins

Note
Deletion of CDKN2A and PAX5 was found in 10 of 10 cases tested, eventhough in cases without isochromosome (9q)
Gene name
ID4 (inhibitor of DNA binding 4, dominant negative helix-loop-helix protein)
Location
6p22.3
Protein description
Member of the ID gene family : "Inhibitors of DNA binding". They are transcription factors which act as transcription inhibitory proteins. They are basic helix-loop-helix (bHLH) proteins which contain the bHLH dimerization domain, but lack the DNA binding domain. They form heterodimers with other bHLH proteins and inhibit them. ID4 may play an important suppressive role in tumor progression, and its silencing by hypermethylation favours tumorogenesis
Gene name
IGH (Immunoglobulin Heavy)
Location
14q32.33

Result of the Chromosomal Anomaly

Description

No hybrid gene, but juxtaposition of ID4 to the IGH enhancer, leading to ID4 overexpression.

Bibliography

Pubmed IDLast YearTitleAuthors

Summary

Fusion gene

IGH/ID4 IGH (14q32.33) ID4 (6p22.3) M t(6;14)(p22;q32)
Atlas Image
t(6;14)(p22;q32) G-banding and FISH - Courtesy Dorothy Hung, Luke St-Heaps and Dale Wrigh, The Cytogenetics Department of Childrens Hospital at Westmead Sydney, Australia.

Citation

Jean-Loup Huret

t(6;14)(p22;q32) IGH/ID4

Atlas Genet Cytogenet Oncol Haematol. 2008-04-01

Online version: http://atlasgeneticsoncology.org/haematological/1480/js/web-card-haematological.js