t(6;14)(q25-27;q32) BCL11B/?

2008-07-01 Jean-Loup Huret Affiliation

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

Clinics and Pathology

Disease

Haematological malignancies

Phenotype stem cell origin

The phenotype is variable, and the translocation may be heterogenous at the level of the genes involved; there was one acute lymphoblastic leukaemia (ALL), four biphenotypic acute leukaemias (BAL), two of which with expression of myeloid and T-cell markers, one acute myeloid leukaemia (AML), one chronic T-cell neoplasm, and one chronic lymphocytic leukaemia (B-CLL) Binet stage B.

Epidemiology

Only 8 cases to date (5M/2F); there was 3 children and 4 adults (ages were: 12, 14, 28, 41, 54, 62, ?, ?).

Prognosis

Survival data is available in only 5 cases; patients died: "shortly", at 17 months, 33 mths, 45 mths, and 112 mths after diagnosis.

Genes Involved and Proteins

Note

In only one of the above mentioned cases, BCL11B was detected as being involved in the translocation; the partner is unknown (Bezrookove et al., 2004). The case with BCL11B involvement was a case of M1-AML in a 54 year old male patient who died shortly after diagnosis. The t(6;14) was the sole anomaly. In other cases, data is missing concerning the genes implicated in the translocation.

Gene name

BCL11B (B-cell lymphoma/leukaemia 11B)

Location

14q32.2

Protein description

Kruppel-like zinc finger protein. Expressed in the immune system and in the brain. Regulates thymic differentiation and survival.

Bibliography

Pubmed ID	Last Year	Title	Authors
8780743	1996	Is t(6;14) a non-random translocation in childhood acute mixed lineage leukemia?	Batanian JR et al
15104287	2004	A novel t(6;14)(q25-q27;q32) in acute myelocytic leukemia involves the BCL11B gene.	Bezrookove V et al
18656690	2008	Acute mixed lineage leukemia and a t(6;14)(q25;q32) in two adults.	Georgy M et al
2364166	1990	14q32 translocations are associated with mixed-lineage expression in childhood acute leukemia.	Hayashi Y et al
11920481	2002	Abnormalities of chromosome bands 15q13-15 in childhood acute lymphoblastic leukemia.	Heerema NA et al
2399910	1990	Cytogenetic findings in 21 cases of peripheral T-cell lymphoma.	Inwards DJ et al
16179374	2006	Chromosomal translocations are associated with poor prognosis in chronic lymphocytic leukemia.	Mayr C et al
2758395	1989	Cytogenetics of childhood acute nonlymphocytic leukemia.	Raimondi SC et al