t(3;21)(q26;q22) RUNX1/MECOM in treatment related leukemia
2003-10-01 Jean-Loup Huret Affiliation1.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
Genes Involved and Proteins
Result of the Chromosomal Anomaly
Highly cited references
| Pubmed ID | Year | Title | Citations |
|---|---|---|---|
| 37904382 | 2023 | A primary pediatric acute myelomonocytic leukemia with t(3;21)(q26;q22): A case report. | 19 |
| 38751957 | 2024 | A rare presentation of BCR-ABL1 and RUNX1-MECOM rearrangement in a pediatric patient with acute myeloid leukemia. | 17 |
| 25379409 | 2014 | An unusual case of splenomegaly and increased lactate dehydrogenase heralding acute myeloid leukemia with eosinophilia and RUNX1-MECOM fusion transcripts. | 12 |
| 22950073 | 2012 | Detection of RUNX1-MECOM fusion gene and t(3;21) in a very elderly patient having acute myeloid leukemia with myelodysplasia-related changes. | 12 |
| 32676933 | 2020 | Analytical and Potential Clinical Performance of Oncomine Myeloid Research Assay for Myeloid Neoplasms. | 0 |
| 23806810 | 2013 | Novel real-time polymerase chain reaction assay for simultaneous detection of recurrent fusion genes in acute myeloid leukemia. | 0 |
Article Bibliography
| Pubmed ID | Last Year | Title | Authors |
|---|---|---|---|
| 11921272 | 2002 | 21q22 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.
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
