T-cell prolymphocytic leukemia (T-PLL)

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

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PMID 2738163

Inversions and tandem translocations involving chromosome 14q11 and 14q32 in T-prolymphocytic leukemia and T-cell leukemias in patients with ataxia telangiectasia.

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PMID 1913594

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PMID 8247530

Multiple karyotypic abnormalities in three cases of small cell variant of T-cell prolymphocytic leukemia.

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PMID 7987802

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PMID 9622061

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PMID 8634440

Abnormalities of chromosomes 8, 11, 14, and X in T-prolymphocytic leukemia studied by fluorescence in situ hybridization.

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PMID 9614908

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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.

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MTCP-1: a novel gene on the human chromosome Xq28 translocated to the T cell receptor alpha/delta locus in mature T cell proliferations.

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Biallelic mutations in the ATM gene in T-prolymphocytic leukemia.

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PMID 9334731

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PMID 9573030

A gene on chromosome Xq28 associated with T-cell prolymphocytic leukemia in two patients with ataxia telangiectasia.

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PMID 8152252

Chromosome walking on the TCL1 locus involved in T-cell neoplasia.

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PMID 8415691

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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

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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

External links

COSMIC	Histo = - Site = haematopoietic_and_lymphoid_tissue (COSMIC)
arrayMap (UZH-SIB Zurich)	Topo ( C42) Morph ( 9834/3) -   [auto + random 100 samples .. if exist ]   [tabulated segments]

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