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3q21q26 rearrangements (RPN1/MECOM) in treatment related leukemia

Written2005-05Jean-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 1236
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-AML)
Note The study included 17 cases; t-MDS without progression to AML accounted for 18%, t-MDS with progression to AML for 18% and t-AML for the remaining 64% (M2 or M4 mainly); no case of acute lymphoblastic leukaemia
Epidemiology 3q21q26 rearrangements were found in 3% of t-MDS/t-AML; sex ratio: 4M/13F
Clinics Age at diagnosis of the primary disease 40 yrs (range 18-69); age at diagnosis of the t-MDS/t-AML: 51 yrs (range 22-80). Median interval was 104 mths (range: 48-217). Primary disease was a solid tumor in 47% of cases and a hematologic malignancy in 53% ( Hodgkin disease and non Hodgkin lymphoma in particular), treatment was chemotherapy (18%), radiotherapy (29%), or both chemotherapy and radiotherapy (53%). Chemotherapy included topoisomerase II inhibitors in 42% of cases and alkylating agents in 100%.
Prognosis Median survival was 7 mths, with 30% of patients surviving at 1 yr, and 20% at 2 yrs.

Cytogenetics

Additional anomalies 3q21q26 rearrangements included inv(3)(q21q26) in 71% of cases, t(3;3)(q21q26)in 23%, and ins(3)(q26;q21q26) in 1 case (6%); additional anomalies were: -7/del(7q) in 82%, -5/del(5q) in 35 %, del(6q) and del(20q) in 12 % each. Complex karyotypes were found in 53%.

Genes involved and Proteins

Gene Name MECOM
Location 3q26
Gene Name RPN1
Location 3q21

Result of the chromosomal anomaly

Hybrid gene
Description RPN1enhancer juxtaposed to EVI1
  

To be noted

Additional cases are needed to delineate the epidemiology of this rare entity:
you are welcome to submit a paper to our new Case Report section.

Bibliography

Rare recurring balanced chromosome abnormalities in therapy-related myelodysplastic syndromes and acute leukemia: report from an international workshop.
Block AW, Carroll AJ, Hagemeijer A, Michaux L, van Lom K, Olney HJ, Baer MR
Genes, chromosomes & cancer. 2002 ; 33 (4) : 401-412.
PMID 11921274
 

Citation

This paper should be referenced as such :
Huret, JL
3q21q26 rearrangements in treatment related leukemia
Atlas Genet Cytogenet Oncol Haematol. 2005;9(3):234-235.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/3q21q26TreatRelLeukID1236.html


External links

arrayMapTopo ( C42) Morph ( 9920/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
 
 
Disease database3q21q26 rearrangements (RPN1/MECOM) in treatment related leukemia
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