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

2007-07-01   Jean-Loup Huret 

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

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

Genes Involved and Proteins

Note
The partner of EVI1 is yet unknown.
Gene name
MECOM (Ecotropic Viral Integration Site 1 (EVI1) and Myelodysplastic Syndrome 1 (MDS1-EVI1)
Location
3q26.2
Protein description
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

Bibliography

Pubmed IDLast YearTitleAuthors
123933832003High EVI1 expression predicts poor survival in acute myeloid leukemia: a study of 319 de novo AML patients.Barjesteh van Waalwijk van Doorn-Khosrovani S et al
119603922002Structural rearrangements of chromosome 3 in 57 patients with acute myeloid leukemia: clinical, hematological and cytogenetic features.Charrin C et al
67227531984Translocation t(3;17)(q26;q22): a marker of acute disease in myeloproliferative disorders?Mecucci C et al
15917131992New case of t(3;17)(q26;q22) as an additional change in a Philadelphia-positive chronic myelogenous leukemia in acceleration.Mugneret F et al
75794621995Different genetic pathways in leukemogenesis for patients presenting with therapy-related myelodysplasia and therapy-related acute myeloid leukemia.Pedersen-Bjergaard J et al
163421722006EVI1 is consistently expressed as principal transcript in common and rare recurrent 3q26 rearrangements.Poppe B et al
175071832007The oncogene and developmental regulator EVI1: expression, biochemical properties, and biological functions.Wieser R et al

Summary

Location

pour K-PRONE
Atlas Image
t(3;17)(q26;q22) G- banding - Courtesy Melanie Zenger and Claudia Haferlach.

Citation

Jean-Loup Huret

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

Atlas Genet Cytogenet Oncol Haematol. 2007-07-01

Online version: http://atlasgeneticsoncology.org/haematological/1282/t(3;17)(q26;q22)

External Links