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T-cell prolymphocytic leukemia (T-PLL)

Written1997-10Lucienne Michaux
Department of Hematology and Center for Human Genetics Cliniques Universitaires Saint Luc Avenue Hippocrate 10 1200 Brussels, Belgium
Updated1999-10Martin Yuille
Academic Department of Haematology, Cytogenetics Institute of Cancer Research, 15 Cotswold Road, SUTTON Surrey SM2 5NG, UK

(Note : for Links provided by Atlas : click)

Identity

ICD-Topo C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
ICD-Morpho 9834/3 T-cell prolymphocytic leukaemia
Atlas_Id 2042

Clinics and Pathology

Disease chronic T-cell lymphoproliferative syndrome
Phenotype / cell stem origin disease affecting mature T-cells; T-cell prolymphocytes usually express CD3, CD5 and CD7; they have either a T-helper (CD4+/CD8-) or a T-suppressor (CD4-/CD8+) phenotype; a small number of cases may co-express CD4 and CD8; this finding is more prevalent in the small cell variant of T-PLL than in classic T-PLL
Epidemiology very rare disease; represents 20% of prolymphocytic leukemias; the disease occurs at advanced age, typically in the 7th or 8th decade; slight male predominance
Clinics splenomegaly is common; lymphadenopathy at presentation is unusual but more frequent than in B-PLL; blood data: high leucocyte counts usually exceeding 100x109/l; T-cell prolymphocytes have the same morphologic features than B-cell prolymphocytes; a small cell variant of T-PLL has been described
Prognosis evolution: progresses rapidly and is generally more aggressive than B-PLL; prognosis: poor response to chemotherapy is observed; median survival is approximatively 7 months from diagnosis

Cytogenetics

Cytogenetics Morphological few cases have been reported in the literature
  • so far; karyotypes are usually complex
  • 14q11 abnormalities: very frequent, either as an inv(14)(q11q32) or as a translocation t(14;14)(q11;q32); another reported change involving 14q11 is a translocation t(X;14)(q28;q11), similar to the translocation observed in ataxia-telangectasia, involving the Mature T-cell Prolymphocyte 1 (MTCP1) gene located at Xq28
  • other recurrent changes involve chromosome 8 either as i(8)(q10) or as der(8) t(8;8)
  • finally, some aberrations involving 12p have been reported
  • Genes involved and Proteins

    Note
  • as with other T-cell neoplasms, T-PLL exhibits clonal rearrangement of T-cell receptor genes; translocation t(X;14)(q28;q11) may result into fusion of MTCP1 with TRA/Dgenes; finally, the TCL1 locus on chromosome 14q32 might also been involved
  • in Ataxia Telangiectasia- a rare recessive pleiotropic disease (including elevated cancer predisposition) mapping to 11q23 and caused by mutations of theATM gene - a recurrent malignancy is observed that is similar to T-PLL; its frequency in A-T patients is higher than in the non-A-T related form; A-T related TPLL has a similar course, a similar immunophenotype and similar cytogenetics (with the notable exception that 11q23 breakpoints are recurrent in the sporadic but not the A-T related form of the disease); an initial report of ATM mutations in T-PLL demonstrated the principle that ATM was a candidate cancer gene in sporadic forms of malignancies prevalent in A-T; the identification of lesions in ATM associated with T-PLL has shown that:
  • homozygous truncating mutations are present in some cases; this suggests ATM can appear to act like a conventional tumour suppressor with biallelic inactivation in the tumour cell
  • missense mutations cluster in the carboxy-terminal phosphatidyl-3-kinase (PIK) domain; this suggests impairment of this domain can contribute to - and may constitute a distinct step in - tumourigenesis
  • rearrangement of the gene is frequent; some rearrangements are consistent with a translocation event, in agreement with cytogenetic data implicating 11q23 in T-PLL; others involve transposition of a segment of the ATM gene elsewhere in the genome.
  • one allele only is mutated (by rearrangement) in some cases; this is probably not associated with a concomitant epigenetic event such as abnormal promoter methylation
  • no T-PLL case has been reported with germline ATM mutation; this may reflect the small numbers investigated; all the same, the hypothesis is excluded that this rare disease is due solely to germline ATM mutation
  • Bibliography

    Proposals for the classification of chronic (mature) B and T lymphoid leukaemias. French-American-British (FAB) Cooperative Group.
    Bennett JM, Catovsky D, Daniel MT, Flandrin G, Galton DA, Gralnick HR, Sultan C
    Journal of clinical pathology. 1989 ; 42 (6) : 567-584.
    PMID 2738163
     
    Inversions and tandem translocations involving chromosome 14q11 and 14q32 in T-prolymphocytic leukemia and T-cell leukemias in patients with ataxia telangiectasia.
    Brito-Babapulle V, Catovsky D
    Cancer genetics and cytogenetics. 1991 ; 55 (1) : 1-9.
    PMID 1913594
     
    The chromosomal translocation t(X;14)(q28;q11) in T-cell pro-lymphocytic leukaemia breaks within one gene and activates another.
    Fisch P, Forster A, Sherrington PD, Dyer MJ, Rabbitts TH
    Oncogene. 1993 ; 8 (12) : 3271-3276.
    PMID 8247530
     
    Multiple karyotypic abnormalities in three cases of small cell variant of T-cell prolymphocytic leukemia.
    Heinonen K, Mahlamäki E, Hämä E, Nousiainen T, Mononen I
    Cancer genetics and cytogenetics. 1994 ; 78 (1) : 28-35.
    PMID 7987802
     
    Ataxia-telangiectasia and T-cell leukemias: no evidence for somatic ATM mutation in sporadic T-ALL or for hypermethylation of the ATM-NPAT/E14 bidirectional promoter in T-PLL.
    Luo L, Lu FM, Hart S, Foroni L, Rabbani H, Hammarström L, Yuille MR, Catovsky D, Webster AD, Vorechovský I
    Cancer research. 1998 ; 58 (11) : 2293-2297.
    PMID 9622061
     
    Expression of p13MTCP1 is restricted to mature T-cell proliferations with t(X;14) translocations.
    Madani A, Choukroun V, Soulier J, Cacheux V, Claisse JF, Valensi F, Daliphard S, Cazin B, Levy V, Leblond V, Daniel MT, Sigaux F, Stern MH
    Blood. 1996 ; 87 (5) : 1923-1927.
    PMID 8634440
     
    Abnormalities of chromosomes 8, 11, 14, and X in T-prolymphocytic leukemia studied by fluorescence in situ hybridization.
    Maljaei SH, Brito-Babapulle V, Hiorns LR, Catovsky D
    Cancer genetics and cytogenetics. 1998 ; 103 (2) : 110-116.
    PMID 9614908
     
    Clinical and laboratory features of 78 cases of T-prolymphocytic leukemia.
    Matutes E, Brito-Babapulle V, Swansbury J, Ellis J, Morilla R, Dearden C, Sempere A, Catovsky D
    Blood. 1991 ; 78 (12) : 3269-3274.
    PMID 1742486
     
    Trisomy 8q due to i(8q) or der(8) t(8;8) is a frequent lesion in T-prolymphocytic leukaemia: four new cases and a review of the literature.
    Mossafa H, Brizard A, Huret JL, Brizard F, Lessard M, Guilhot F, Tanzer J
    British journal of haematology. 1994 ; 86 (4) : 780-785.
    PMID 7918072
     
    Cytogenetic findings in peripheral T-cell lymphomas as a basis for distinguishing low-grade and high-grade lymphomas.
    Schlegelberger B, Himmler A, Gödde E, Grote W, Feller AC, Lennert K
    Blood. 1994 ; 83 (2) : 505-511.
    PMID 8286748
     
    MTCP-1: a novel gene on the human chromosome Xq28 translocated to the T cell receptor alpha/delta locus in mature T cell proliferations.
    Stern MH, Soulier J, Rosenzwajg M, Nakahara K, Canki-Klain N, Aurias A, Sigaux F, Kirsch IR
    Oncogene. 1993 ; 8 (9) : 2475-2483.
    PMID 8361760
     
    Biallelic mutations in the ATM gene in T-prolymphocytic leukemia.
    Stilgenbauer S, Schaffner C, Litterst A, Liebisch P, Gilad S, Bar-Shira A, James MR, Lichter P, Döhner H
    Nature medicine. 1997 ; 3 (10) : 1155-1159.
    PMID 9334731
     
    Inactivation of the ATM gene in T-cell prolymphocytic leukemias.
    Stoppa-Lyonnet D, Soulier J, Laugé A, Dastot H, Garand R, Sigaux F, Stern MH
    Blood. 1998 ; 91 (10) : 3920-3926.
    PMID 9573030
     
    A gene on chromosome Xq28 associated with T-cell prolymphocytic leukemia in two patients with ataxia telangiectasia.
    Thick J, Mak YF, Metcalfe J, Beatty D, Taylor AM
    Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K. 1994 ; 8 (4) : 564-573.
    PMID 8152252
     
    Chromosome walking on the TCL1 locus involved in T-cell neoplasia.
    Virgilio L, Isobe M, Narducci MG, Carotenuto P, Camerini B, Kurosawa N, Abbas-ar-Rushdi, Croce CM, Russo G
    Proceedings of the National Academy of Sciences of the United States of America. 1993 ; 90 (20) : 9275-9279.
    PMID 8415691
     
    Clustering of missense mutations in the ataxia-telangiectasia gene in a sporadic T-cell leukaemia.
    Vorechovský I, Luo L, Dyer MJ, Catovsky D, Amlot PL, Yaxley JC, Foroni L, Hammarström L, Webster AD, Yuille MA
    Nature genetics. 1997 ; 17 (1) : 96-99.
    PMID 9288106
     
    ATM is usually rearranged in T-cell prolymphocytic leukaemia.
    Yuille MA, Coignet LJ, Abraham SM, Yaqub F, Luo L, Matutes E, Brito-Babapulle V, Vorechovský I, Dyer MJ, Catovsky D
    Oncogene. 1998 ; 16 (6) : 789-796.
    PMID 9488043
     

    Citation

    This paper should be referenced as such :
    Yuille, M
    T-cell prolymphocytic leukemia (T-PLL)
    Atlas Genet Cytogenet Oncol Haematol. 1999;3(4):187-188.
    Free journal version : [ pdf ]   [ DOI ]
    On line version : http://AtlasGeneticsOncology.org/Anomalies/TPLLID2042.html
    History of this paper:
    Michaux, L. T-cell prolymphocytic leukemia (T-PLL). Atlas Genet Cytogenet Oncol Haematol. 1997;1(2):83-84.
    http://documents.irevues.inist.fr/bitstream/handle/2042/32064/10-1997-TPLLID2042.pdf


    Other genes implicated (Data extracted from papers in the Atlas) [ 8 ]

    Genes MTCP1 SOX11 SOX4 TCL1B TCL1A TCL1A TRA TRD

    External links

    COSMICHisto = - Site = haematopoietic_and_lymphoid_tissue (COSMIC)
    arrayMapTopo ( C42) Morph ( 9834/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
     
     
    Disease databaseT-cell prolymphocytic leukemia (T-PLL)
    REVIEW articlesautomatic search in PubMed
    Last year articlesautomatic search in PubMed
    All articlesautomatic search in PubMed


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