del(13q) in ALL

2004-11-01   David Betts 

1.Department of Oncology, University Childrens Hospital, Steinwiesstr. 75, CH-8032 Zò_rich, Switzerland

Clinics and Pathology

Disease

Acute lymphoblastic leukaemia (ALL)

Phenotype stem cell origin

No specific immunophenotype observed

Epidemiology

A del(13q) chromosome is found in approximately 2% of cases in both adult and childhood disease at presentation. Up to 4% of cases may have some loss of 13q material, either through full monosomy or unbalanced rearrangements. Incidence of chromosome 13 deletions is higher at relapse.

Prognosis

May confer an increased risk of treatment failure but to date has not been shown to be an independent prognostic indicator.

Cytogenetics

Cytogenetics morphological

Various breakpoints reported. The centromeric breakpoint is typically in the 13q12-14 region and telomeric between 13q21 and 13qter. Loss of all or part of 13q14 is common to almost all cases. Occurs as a sole event in approximately 10% of cases. There are also rare reports of translocations also leading to a partial 13q deletion. Monosomy 13 is also reported but occurs very rarely as a sole aberration. Under representation of chromosome 13 is often found in hypotriploid cases.

Additional anomalies

Most cases with del(13q) will have additional aberrations, but there is no consistent picture and the events can include the typical non-random events in ALL.

Genes Involved and Proteins

Note
Critical region in 13q14 appears to lie telomeric to RB1.

Bibliography

Pubmed IDLast YearTitleAuthors
112370592001Deletion of chromosomal region 13q14.3 in childhood acute lymphoblastic leukemia.Cavé H et al
107176172000Deletions in the 13q14 locus in adult lymphoblastic leukemia: rate of incidence and relevance.Chung CY et al
110784972000Abnormalities of chromosome bands 13q12 to 13q14 in childhood acute lymphoblastic leukemia.Heerema NA et al
151727542004Aberrations involving 13q12 approximately q14 are frequent secondary events in childhood acute lymphoblastic leukemia.Kovacs BZ et al
110019092000New recurring cytogenetic abnormalities and association of blast cell karyotypes with prognosis in childhood T-cell acute lymphoblastic leukemia: a pediatric oncology group report of 343 cases.Schneider NR et al

Summary

Note

Deletions of chromosome 13q are a non-random finding in a broad spectrum of haematological neoplasms, including B-cell chronic lymphocytic leukemia (CLL), non-Hodgkins lymphoma (NHL) and multiple myeloma (MM) and (AML)

Citation

David Betts

del(13q) in ALL

Atlas Genet Cytogenet Oncol Haematol. 2004-11-01

Online version: http://atlasgeneticsoncology.org/haematological/1188/del(13q)-in-all

External Links