Cancer Report

Cancer : t(9;22)(q34;q11) in ALL
Summary
Cancer Name t(9;22)(q34;q11) in ALL
Description Although the same hybrid genes issued from ABL and BCR are the hallmark of the t(9;22) translocation, this translocation may be seen in the following diseases: CML, ANLL, and ALL, and will therefore be described in the 3 different situations: t(9;22)(q34;q11) in CML, t(9;22)(q34;q11) in ALL, t(9;22)(q34;q11) in ANLL t(9;22)(q34;q11) in ALL is herein described.
 
Clinics and Pathology
Disease ALL
Epidemiology 20% of adult ALL, 2-5% of children ALL
Treatment BMT is indicated
Prognosis is very poor, especially in lymphoid-committed progenitor cases; the breakpiont in M-bcr or in m-bcr (see below) does not seem to have impact on prognosis.
 
Related Genes
Gene Symbol ABL1
Description most often CD 10+ ALL; frequent CNS involvement
 
Gene Symbol BCR
Description " various splicingsmain form: 160 KDa; N-term Serine-Treonine kinase domain, SH2 binding, and C-term domain which functions as a GTPase activating protein for p21rac; widely expressed; cytoplasmic localisation; protein kinase; probable role in signal transduction "
 
Cytogenetics
Cytogenetics Molecular is useful to uncover a 'masked Philadelphia' chromosome, where chromosomes 9 and 22 all appear to be normal, but where cryptic insertion of 3' ABL within a chromosome 22 can be demonstrated
 
Bibliography
Title The function of BCR/ABL and related proto-oncogenes.
Authors Gotoh A, Broxmeyer HE
Citations Curr Opin Hematol. 1997 Jan;4(1):3-11. Review.
PubMedID 97196352
 
Title The molecular pathology of chronic myelogenous leukaemia.
Authors Kurzrock R, Talpaz M
Citations Br J Haematol. 1991 Oct;79 Suppl 1:34-7. Review.
PubMedID 92031068
 
Title Molecular insights into the Philadelphia translocation.
Authors Heisterkamp N, Groffen J
Citations Hematol Pathol. 1991;5(1):1-10. Review.
PubMedID 91267879
 
Title Prognostic implications of breakpoint and lineage heterogeneity in Philadelphia-positive acute lymphoblastic leukemia: a review.
Authors Secker-Walker LM, Craig JM
Citations Leukemia. 1993 Feb;7(2):147-51. Review.
PubMedID 93148664
 
Source and Citation
Source Atlas of Genetics and Cytogenetics in Oncology and Haematology
Citation Huret JL . t(9;22)(q34;q11) in ALL. Atlas Genet Cytogenet Oncol Haematol. September 1997 .
URL : http://AtlasGeneticsOncology.org/Anomalies/t0922ALL.html
URL http://AtlasGeneticsOncology.org/Anomalies/t0922ALL.html