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t(3;8)(p25;q24)

Written2018-08Jean-Loup Huret
Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France

Abstract Review on t(3;8)(p25;q24), with data on clinics

Keywords Chromosome 3; chromosome 8; Blastic plasmacytoid dendritic cell neoplasm

(Note : for Links provided by Atlas : click)

Identity

ICD-Topo C420,C421,C424
ICD-Morpho 9727/3 Blastic plasmacytoid dendritic cell neoplasm
Atlas_Id 1827

Clinics and Pathology

Disease Blastic plasmacytoid dendritic cell neoplasm (BPDCN)
Note BPDCN has been known with various names, including agranular CD4+ natural killer (NK) leukemia, CD4+/CD56+ hematodermic neoplasm, and blastic NK lymphoma. BPDCN malignant cells are derived from the precursors of plasmacytoid dendritic cells. It most commonly involves the skin. BPDCN is an aggressive neoplasm. BPDCN is often associated with a complex karyotype (review in Meloni-Ehrig 2017).
Epidemiology In a series of 41 patients with BPDCN, five had a MYC rearrangement confirmed by FISH, one had a t(X;8)(q24;q24), one had a t(3;8)(p25;q24), two had a t(6;8)(p21;q24) MYC/SUPT3H, and one had a t(8;14)(q24.1;q32) (Boddu et al., 2018).
Clinics The patient with a t(3;8)(p25;q24) was a 66 year-old male patient with skin, lymph nodes and central nervous system involvement. He was alive and well 12 months+ after diagnosis.
Cytogenetics The karyotype was complex.

Genes involved and Proteins

Note The partner gene of MYC is unknown.
Gene NameMYC
Location 8q24.21
Dna / Rna MYC is composed of three exons spanning over 4 kb.
Protein MYC is expressed in almost all proliferating cells. It is located predominantly in the nucleus. MYC is a transcriptional regulator, capable to induce or repress the expression of thousands genes. MYC is deregulated in cancer by several different mechanisms: chromosomal translocations, amplifications, point mutations, epigenetic reprogramming, enhanced translation and increased protein stability (review in Mohamed, 2017).

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

8q24/MYC rearrangement is a recurrent cytogenetic abnormality in blastic plasmacytoid dendritic cell neoplasms.
Boddu PC, Wang SA, Pemmaraju N, Tang Z, Hu S, Li S, Xu J, Medeiros LJ, Tang G.
Leuk Res. 2018 Mar;66:73-78.
PMID 29407586
 
Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN)
Meloni-Ehrig A
Atlas Genet Cytogenet Oncol Haematol. in press http://AtlasGeneticsOncology.org/Anomalies/BPDCNID2146.html
 
MYC (MYC proto-oncogene, bHLH transcription factor);
Mohamed AN
Atlas Genet Cytogenet Oncol Haematol. in press http://AtlasGeneticsOncology.org/Genes/MYCID27.html
 

Citation

This paper should be referenced as such :
Jean-Loup Huret
t(3;8)(p25;q24)
Atlas Genet Cytogenet Oncol Haematol. 2018;22(12):518-519.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0308p25q24ID1827.html


Translocations implicated (Data extracted from papers in the Atlas)

 t(3;8)(p25;q24) ?/MYC

External links

Mitelman databaset(3;8)(p25;q24) [Case List]    t(3;8)(p25;q24) [Transloc-MCList] ?/MYC [Fusion-MCList]
arrayMap (UZH-SIB Zurich)Morph ( 9727/3) -   [auto + random 100 samples .. if exist ]   [tabulated segments]
 
 
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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indexed on : Wed Nov 28 16:18:11 CET 2018


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For comments and suggestions or contributions, please contact us

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