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

Written2003-10Jean-Loup Huret
Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France

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Identity

ICD-Topo C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
ICD-Morpho 9920/3 Therapy-related myeloid neoplasms
Atlas_Id 1294
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.
 
  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.

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

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.
  

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
 

Citation

This paper should be referenced as such :
Huret, JL
t(3;21)(q26;q22) in treatment related leukemia
Atlas Genet Cytogenet Oncol Haematol. 2004;8(1):22-23.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0321q26q22TreatRelID1294.html


Translocations implicated (Data extracted from papers in the Atlas)

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

External links

RUNX1 (21q22.12) MECOM (3q26.2)

RUNX1 (21q22.12) MECOM (3q26.2)

Mitelman databaset(3;21)(q26;q22) [Case List]    t(3;21)(q26;q22) [Association List] Mitelman database (CGAP - NCBI)
arrayMapTopo ( C42) Morph ( 9920/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
 
Mitelman databaseRUNX1/MECOM [MCList]  RUNX1 (21q22.12) MECOM (3q26.2)
TCGA_FusionRUNX1/MECOM [LAML]  RUNX1 (21q22.12) MECOM (3q26.2)
 
Disease databaset(3;21)(q26;q22) RUNX1/MECOM in treatment related leukemia
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