Cancer Report

Cancer : inv(14)(q11q32.1)
Cancer Name inv(14)(q11q32.1)
Clinics and Pathology
Disease T Prolymphocytic leukemia (T-PLL)
Prognosis T-PLL has an aggressive clinical course, although, in a small proportion on cases, disease evolves with a slowly progressive lymphocytosis (phenotype CD45RO-CD45RA-)
Disease Ataxia telangiectasia (AT)
Disease Adult T-cell leukemia/lymphoma
Disease Acute lymphoblastic leukemia (ALL) of T lineage
Disease Leukemias of B lineage
Disease Acute myeloid leukemia with lymphoid associated antigens
Related Genes
Gene Symbol CLTCL1
Description "
  • TCL1B is located on 14q32.1 16 Kb centromeric of TCL1A and shows 60% similarity to TCL1; TCL1A and TCL1B are located in the about 160 kb region of breakpoints observed in T cell leukemia cases with translocations at 14q32.1
  • Semi quantitative RT-PCR analysis revealed that both TCL1A and TCL1b genes are expressed in spleen, tonsil, fetal liver, fetal kidney and fetal thymus. However the TCL1B gene is expressed in a wide variety of tissues. Normally, TCL1A expression is observed in early T cell progenitors (CD4- CD8- CD3-) and lymphoid cell of the B lineage : pre B cells and immature IgM expressing B cells.
  • TCL1A, TCL1B encode for protein of about 14 kDa. TCL1A 14 kDa protein consists of an eight-stranded antiparallel beta barrel with a hydrophobic core and are predicted to bind small hydrophobic ligands such as retinoids, nucleosides or fatty acids.
  • NOTE : in addition to TCL1 and TCL1b the locus contains two additional genes TCL1- neighboring gene (TNG1 and TNG2) encoding proteins of 141 and 110 amino acids. Recently two novel genes have been identified in this region : TML1 and TCL6. Since the TCL1, TCL1b, TML1, TCL6 genes are expressed in T-cell leukemias carrying a t(14;14) translocation, they are four candidate genes potentially involved in leukemogenesis. "
    Gene Symbol MTCP1
    Description The MTCP1 is located at Xq28 and activated in rare cases of T-PLL with a t(X;14)(q28;q11) translocation.MTCP1 encodes for two proteins p8MTCP1 and p13MTCP1
    Gene Symbol TCL1A
    Description "TRA@ - TCL1 in T-cell malignancies. mainly, T-cell prolymphocytic leukemia (T-PLL); T- cell NHL; T-cell ALL "
    Gene Symbol TRD
    Description "
  • The size of TCR alpha/delta locus is about 1 Mb. The TCR delta variable (V) diversity (D) joining (J) and constant region genes are situated within the TCR alpha locus between the TCR alpha V and the TCR alpha J segments.
  • The TCR delta locus contains three D segments and four J segments, whereas the TCR alpha J regions spans approximately 80 Kb and contains at least 61 segments.
  • The TCR alpha/delta locus is transcribed in a centromer to telomer direction.T-cell receptor "
    Title A complex pattern of recurrent chromosomal losses and gains in T-cell prolymphocytic leukemia
    Authors Soulier J, Pierron G, Vecchione D, Garand R, Brizard F, Sigaux F, Stern MH, Aurias A
    Citations Genes Chromosomes Cancer 2001 Jul ; 31 (3) : 248-254
    PubMedID 12759932
    Title Abnormality at 14q32.1 in T cell malignancies involve two oncogenes
    Authors Pekarsky Y, Hallas C, Isobe M, Russo G, Croce CM
    Citations Proc Natl Acad Sci USA 1999 96 (6) : 2949-2951
    PubMedID 12759932
    Title Abnormalities of the short arm of chromosome 12 in T cell prolymphocytic laukemia.
    Authors Salomon-Nguyen F, Brizard F, Le Coniat M, Radford I, Berger R, Brizard A
    Citations Leukemia 1998 Jun; 12 (6) : 972-975
    PubMedID 12759932
    Title Concurrent presence of inv(14)(q11;q32) and t(4;11)(q21;q23) in pre-B acute lymphoblastic leukemia.
    Authors Chervinsky DS, Grossi M, Kakati S, Block AW, Aplan PD
    Citations Genes Chromosomes Cancer 1995 Mar; 12 (3) : 229-236
    PubMedID 12759932
    Title Clinical and laboratory features of 78 cases of T-prolymphocytic leukemia.
    Authors Matutes E, Brito-Babapulle V, Swansbury J et al
    Citations Blood 1991 ; 78 : 3269-3274
    PubMedID 12759932
    Title Genomic analysis of human and mouse TCL1 loci reveals a complex of tightly clustered genes
    Authors Hallas C, Pekarsky Y, Itoyama T, Varnum J, Bichi R, Rothstein JL, Croce CM
    Citations Proc Natl Acad Sci USA 1999 Dec; 96 (25) : 14418-14423
    PubMedID 12759932
    Title Cytogenetic studies on prolymphocytic leukemia
    Authors Brito-Babapulle V, Pomfret M, Matutes E, et al
    Citations Blood 1987; 70 : 926-931
    PubMedID 12759932
    Title Rearrangements in the T-cell receptor alpha chain locus in patients with adult T-cell leukemia carrying translocations involving chromosome 14q11.
    Authors Isobe M, Sadamori N, Russo G, Shimizu S, Yamamori S, Itoyama T, Yamada Y, Ikeda S, Ichimaru M, Kagan J, et al.
    Citations Cancer Res 1990 Oct 1;50 (19) : 6171-5
    PubMedID 12759932
    Title Significance of chromosome 14 anomaly at band 14q11 in Japanese Patients with Adult T-cell leukemia
    Authors Sadamori N, Nishino K, Kusano M et al
    Citations Cancer 1986; 58 : 2244-2250
    PubMedID 12759932
    Title The protooncogene TCL1 is an Akt kinase coactivator
    Authors Laine J, Kunstle G, Obata T, Sha M, Noguchi M
    Citations Mol Cell 2000 Aug; 6 (2) : 395-407
    PubMedID 12759932
    Title Trisomy 8q due to iso(8q) or der (8) t(8;8) is a frequent lesion in T-prolymphocytic leukemia : four new cases and a rewiew of the literature
    Authors Mossafa H, Brizard A, Huret JL, Brizard F, Lessart M, Guilhot F, Tanzer J
    Citations Br J Haematol 1994 Apr ; 86 (4) : 780-785
    PubMedID 12759932
    Title Identification of the TCL1 gene involved in T-cell malignancies
    Authors Virgilio l, Narducci mg, Isobe M, Billips LG, Cooper MD, Croce CM, Russo G
    Citations Proc Natl Acad Sci USA 1994 Dec; 91 (26) : 12530-12534
    PubMedID 12759932
    Title Identification of the TCL1/MTCP1 like 1 (TML1) gene from the region next to the TCL1 locus
    Authors Sugimoto J, Hatakeyama T, Narducci MG, Russo G, Isobe M
    Citations Cancer Research 1999 May; 59 : 2313-2317
    PubMedID 12759932
    Title Identification of the TCL6 genes within the breakpoint cluster region on chromosome 14q32 in T-cell leukemia
    Authors Saitou M, Sugimoto J, Hatakeyama T, Russo G, Isobe M
    Citations Oncogene 2000 May ; 19 (23) : 2796-2802
    PubMedID 12759932
    Title Inversion (14) q11;q32) in chilhood T-cell acute lymphoblastic leukemia.
    Authors Kwong YL, Shing MK, Wan TM, Yuen PM, Chan AY, Wong KF, Chan LC
    Citations Cancer Genet Cytogenet 1994 Feb; 72 (2) : 92-95
    PubMedID 12759932
    Title Inversion (14)(q11;q32) in a case of acute myeloid leukemia expressing lymphoid-associated antigens.
    Authors Raynaud SD, Brunet B, Ayraud N, Monpoux F, Philip P, Bayle J
    Citations Cancer Genet Cytogenet 1993 Nov; 71(1) : 100-101
    PubMedID 12759932
    Title Inversion 14q in acute lymphoblastic leukemia of B-lineage
    Authors Wong KF, Kwong YL, Wong TK
    Citations Cancer Genet Cytogenet 1995 Mar; 80 (1) : 72-74
    PubMedID 12759932
    Title Karyotype and T-cell receptor expression in T-lineage acute lymphoblastic leukemia.
    Authors Secker-Walker LM, Campana D, Hawkins JM, Sampson RE, Coustan-Smith E.
    Citations Genes Chromosomes Cancer 1992 Jan; 4(1) : 41-5
    PubMedID 12759932
    Title Molecular cloning of a DNA fragment from human chromosom 14(14q11) involved in T-cell malignancies.
    Authors Mathieu-Mahul D, Caubet JF, Bernheim A, Mauchauffe M, Palmer E, Berger R, Larsen CJ
    Citations EMBO J 1985 Dec 16;4 (13A) : 3427-33
    PubMedID 12759932
    Source and Citation
    Source Atlas of Genetics and Cytogenetics in Oncology and Haematology
    Citation Boyer J . inv(14)(q11q32.1),t(14;14)(q11;q32.1). Atlas Genet Cytogenet Oncol Haematol. June 2001 .
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