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t(3;17)(q26;q22) ?/MECOM

Written2007-07Jean-Loup Huret
Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France

(Note : for Links provided by Atlas : click)

Identity

ICD-Topo C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
ICD-Morpho 9861/3 AML with mutated NPM1; AML with mutated CEBPA; Acute myeloid leukaemia, NOS
ICD-Morpho 9920/3 Therapy-related myeloid neoplasms
ICD-Morpho 9975/3 Chronic myelogenous leukaemia, BCR-ABL1 positive; Myeloproliferative neoplasm, unclassifiable; Myelodysplastic/myeloproliferative neoplasm, unclassifiable
Atlas_Id 1282
 
  t(3;17)(q26;q22) G- banding - Courtesy Melanie Zenger and Claudia Haferlach.

Clinics and Pathology

Disease Chronic myelogenous leukaemia with t(9;22)(q34;q11) and blast crisis of CML (BC-CML) (4 cases altogether), other myeloproliferative syndromes, Acute myeloid leukaemia (AML) in 4 cases (one M1, 2 therapy related AML,one of which after breast cancer).
Epidemiology At least 9 cases to date, aged 62 years (medain, range: 49-78); sex ration was 5M/3F.
Prognosis No data

Cytogenetics

Cytogenetics Morphological Sole anomaly in 3 cases, with t(9;22)(q34;q11) in 4 cases (one of which was a complex translocation), with del(5q) (1 case), and with del(7q), and +21 in 1 case.

Genes involved and Proteins

Note The partner of EVI1 is yet unknown.
Gene Name MECOM
Location 3q26.2
Protein Transcrition factor; EVI1 targets include:GATA2, ZBTB16 /PLZF, ZFPM2/FOG2, JNK and the PI3K/AKT pathway. Role in cell cycle progression, likely to be cell-type dependant; antiapoptotic factor; involved in neuronal development organogenesis; role in hematopoietic differsntiation

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

High EVI1 expression predicts poor survival in acute myeloid leukemia: a study of 319 de novo AML patients.
Barjesteh van Waalwijk van Doorn-Khosrovani S, Erpelinck C, van Putten WL, Valk PJ, van der Poel-van de Luytgaarde S, Hack R, Slater R, Smit EM, Beverloo HB, Verhoef G, Verdonck LF, Ossenkoppele GJ, Sonneveld P, de Greef GE, Löwenberg B, Delwel R
Blood. 2003 ; 101 (3) : 837-845.
PMID 12393383
 
Structural rearrangements of chromosome 3 in 57 patients with acute myeloid leukemia: clinical, hematological and cytogenetic features.
Charrin C, Belhabri A, Treille-Ritouet D, Theuil G, Magaud JP, Fiere D, Thomas X
The hematology journal : the official journal of the European Haematology Association / EHA. 2002 ; 3 (1) : 21-31.
PMID 11960392
 
Translocation t(3;17)(q26;q22): a marker of acute disease in myeloproliferative disorders?
Mecucci C, Michaux JL, Broeckaert-Van Orshoven A, Symann M, Boogaerts M, Kulling G, Van den Berghe H
Cancer genetics and cytogenetics. 1984 ; 12 (2) : 111-119.
PMID 6722753
 
New case of t(3;17)(q26;q22) as an additional change in a Philadelphia-positive chronic myelogenous leukemia in acceleration.
Mugneret F, Solary E, Favre B, Caillot D, Sidaner I, Guy H
Cancer genetics and cytogenetics. 1992 ; 60 (1) : 90-92.
PMID 1591713
 
Different genetic pathways in leukemogenesis for patients presenting with therapy-related myelodysplasia and therapy-related acute myeloid leukemia.
Pedersen-Bjergaard J, Pedersen M, Roulston D, Philip P
Blood. 1995 ; 86 (9) : 3542-3552.
PMID 7579462
 
EVI1 is consistently expressed as principal transcript in common and rare recurrent 3q26 rearrangements.
Poppe B, Dastugue N, Vandesompele J, Cauwelier B, De Smet B, Yigit N, De Paepe A, Cervera J, Recher C, De Mas V, Hagemeijer A, Speleman F
Genes, chromosomes & cancer. 2006 ; 45 (4) : 349-356.
PMID 16342172
 
The oncogene and developmental regulator EVI1: expression, biochemical properties, and biological functions.
Wieser R
Gene. 2007 ; 396 (2) : 346-357.
PMID 17507183
 

Citation

This paper should be referenced as such :
Huret, JL
t(3;17)(q26;q22)
Atlas Genet Cytogenet Oncol Haematol. 2008;12(3):246-247.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0317q26q22ID1282.html


Translocations implicated (Data extracted from papers in the Atlas)

 t(3;17)(q26;q22) ?/MECOM

External links

Mitelman databaset(3;17)(q26;q22) [Case List]    t(3;17)(q26;q22) [Association List] Mitelman database (CGAP - NCBI)
arrayMapTopo ( C42) Morph ( 9861/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
arrayMapTopo ( C42) Morph ( 9920/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
arrayMapTopo ( C42) Morph ( 9975/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
 
 
Disease databaset(3;17)(q26;q22) ?/MECOM
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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