t(3;21)(q26;q22) RUNX1/MECOM in treatment related leukemia

2003-10-01   Jean-Loup Huret 

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

Clinics and Pathology

Disease

Treatment related myelodysplasia (t-MDS) or acute myeloid leukaemias (t-AML)

Note

The study included 16 cases; t-MDS without progression to AML accounted for 38%, t-MDS progressing to AML for 25%, t-AML for the remaining 38% (to be compared with the 80% of t-AML 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-AML; 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-AML: 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

Result of the Chromosomal Anomaly

Description

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

Bibliography

Pubmed IDLast YearTitleAuthors
11921272200221q22 balanced chromosome aberrations in therapy-related hematopoietic disorders: report from an international workshop.Slovak ML et al

Summary

Fusion gene

RUNX1/MECOM RUNX1 (21q22.12) MECOM (3q26.2) M t(3;21)(q26;q22)|RUNX1/MECOM RUNX1 (21q22.12) MECOM (3q26.2) TF LAML

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.
Atlas Image
t(3;21)(q26;q22) left: G- banding - top three: Courtesy Melanie Zenger and Claudia Haferlach; bottom three: Courtesy Nathalie Nadal; center: R- banding - top two: Courtesy Peter Meeus; middle two: Courtesy Nathalie Nadal; bottom two: Courtesy Christine Pérot; right: FISH with EVI1 break apart probe Courtesy Marian Stevens-Kroef, and diagram Courtesy Peter Meeus.

Citation

Jean-Loup Huret

t(3;21)(q26;q22) RUNX1/MECOM in treatment related leukemia

Atlas Genet Cytogenet Oncol Haematol. 2003-10-01

Online version: http://atlasgeneticsoncology.org/haematological/1294/t(3;21)(q26;q22)-runx1-mecom-in-treatment-related-leukemia

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