Cancer Report

Cancer : t(9;22)(q34;q11) in CML
Summary
Cancer Name t(9;22)(q34;q11) in CML
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: chronic myelogenous leukemia (CML), acute non lymphocytic leukemia (ANLL), and acute lymphocytic leukemia (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 CML is herein described
 
Clinics and Pathology
Disease CML: all CML have a t(9;22), at least at the molecular level (see below); but not all t(9;22) are found in CML, as already noted
Epidemiology annual incidence: 10/106 (from 1/106 in childhood to 30/106 after 60 yrs); median age: 30-60 yrs; sex ratio: 1.2M/1F
Treatment aIFN therapy or allogeneic bone marrow transplantation (BMT), donor leukocytes infusions
Prognosis median survival: 4 yrs with conventional therapy (hydroxyurea, busulfan), 6 yrs with aIFN therapy; allogeneic bone marrow transplantation may cure the patient; otherwise, the best treatment to date associates interferon a, hydroxyurea and cytarabine
 
Related Genes
Gene Symbol BCR
Description " all CML have a t(9;22), at least at the molecular level (BCR/ABL); phenotype and stem cell origin: multipotent progenitor: t(9;22) is found in all myeloid and B- lineage progenitors "
 
Gene Symbol CEBPA
Description "CEBPA is rarely involved in translocations with other genes. However, in B-cell precursor acute lymphoblastic leukemia (BCP-ALL) the 3'UTR of CEBPA has been found to be translocated to the immunoglobulin heavy chain locus"
 
Gene Symbol ABL1
Description " all CML have a t(9;22), at least at the molecular level (BCR/ABL); phenotype and stem cell origin: multipotent progenitor: t(9;22) is found in all myeloid and B- lineage progenitors "
 
Cytogenetics
Cytogenetics Molecular is a useful tool for diagnostic ascertainment in the case of 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
 
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Source and Citation
Source Atlas of Genetics and Cytogenetics in Oncology and Haematology
Citation Huret JL . t(9;22)(q34;q11) in CML. Atlas Genet Cytogenet Oncol Haematol. December 1997 .
URL : http://AtlasGeneticsOncology.org/Anomalies/t0922CML.html
URL http://AtlasGeneticsOncology.org/Anomalies/t0922CML.html