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t(3;21)(q26;q22) in treatment related leukemia

Identity

Note This data is extracted from a very large study from an International Workshop on treatment related leukemias - restricted to balanced chromosome aberrations (i.e.: -5/del(5q)and -7/del(7q) not taken into account per see), published in Genes,Chromosomes and Cancer in 2002.

Clinics and Pathology

Disease Treatment related myelodysplasia (t-MDS) or acute non lymphocytic leukaemias (t-ANLL)
Note The study included 16 cases; t-MDS without progression to ANLL accounted for 38%, t-MDS progressing to ANLL for 25%, t-ANLL for the remaining 38% (to be compared with the 80% of t-ANLL in cases with t(8;21)); no case of acute lymphoblastic leukaemia
Epidemiology t(3;21)(q26;q22) was found in 3% of t-MDS/t-ANLL; sex ratio: 5M/11F
Clinics Age at diagnosis of the primary disease 49 yrs (range 14-72); age at diagnosis of the t-MDS/t-ANLL: 53 yrs range 19-73). Median interval was 36 mths, range: 17-139). Primary disease was a solid tumor in 56% of cases and a hematologic malignancy in 44%. Treatment included topoisomerase II inhibitors in 81% of cases).
Prognosis Median survival was 8 mths. Outcome was worse than the outcome of patients with t(8;21)(q22;q22), t(15;17) or inv(16) treatment related leukemias, and similar to the outcome of patients with 11q23 rearrangement

Cytogenetics

Additional anomalies The t(3;21) was found solely in 31% of cases; additional anomaly was: -7/del(7q) in 31% of cases, +8 was not observed . A complex karyotype was found in 25% of cases

Result of the chromosomal anomaly

Hybrid gene
Description 5' AML1 - 3' MDS1-EVI1; breakpoint is most often in the AML1 intron 6.
  

External links

Other databaset(3;21)(q26;q22) in treatment related leukemia Mitelman database (CGAP - NCBI)
Other databaset(3;21)(q26;q22) in treatment related leukemia CancerChromosomes (NCBI)

Bibliography

21q22 balanced chromosome aberrations in therapy-related hematopoietic disorders: report from an international workshop.
Slovak ML, Bedell V, Popplewell L, Arber DA, Schoch C, Slater R
Genes, chromosomes & cancer. 2002 ; 33 (4) : 379-394.
PMID 11921272
 

Contributor(s)

Written10-2003Jean-Loup Huret

Citation

This paper should be referenced as such :
Huret JL . t(3;21)(q26;q22) in treatment related leukemia. Atlas Genet Cytogenet Oncol Haematol. October 2003 .
URL : http://AtlasGeneticsOncology.org/Genes/t0321q26q22TreatRelID1294.html

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indexed on : Mon Jul 14 16:32:58 2008


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