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Classification of T-Cell disorders

Written2001-02Vasantha Brito-Babapulle, Estella Matutes, Daniel Catovsky
Academic Department of Haematology, Cytogenetics, The Royal Marsden NHS Trust, London, UK

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ICD-Morpho 9591/3 Splenic B-cell lymphoma/leukaemia, unclassifiable; Splenic diffuse red pulp small B-cell lymphoma; Hairy cell leukaemia-variant
Atlas_Id 2079
Note T-cell lymphoid disorders include a variety of disease entities which result from the clonal neoplastic expansion of an uncommitted (thymic) or a committed (post thymic) T-cell. Some of these diseases have distinct cytogenetic/molecular genetic features which allow to better define the various entities and understand their pathogenesis.

Clinics and Pathology

Disease T-prolymphocytic leukemia (T-PLL)
Variants: small cell and cerebriform cell.
Phenotype / cell stem origin TdT-, CD1a-,
CD4+ CD8-
CD4- CD8+
CD4+ CD8+
Clinics Aggressive course
Splenomegaly, high WBC with prolymphocytes.
Cytogenetics inv(14)(q11q32), t(14;14)(q11;q32)
Xq28 abnormalities
idic(8)(p11), t(8;8)(p11;q1-2)
11q22-23 abnormalities
12p abnormalities
13q14.3 deletions
Genes ATM gene (11q22-23) mutated.
TCL1 (14q32.1) or
MTCP1 (Xq28) activated.

Disease Large granular lymphocyte leukemia (LGL) - T-cell Type
Phenotype / cell stem origin TdT-, CD1a
CD3+, CD2+, CD8+ CD4-, CD57+, CD16+/-Cytotoxic or suppressor activity.
Clinics Indolent
Cytopenias, splenomegaly, lymphocytosis with granular lymphocytes.
Cytogenetics Clonal abnormalities. In some cases, but no consistent specific abnormalities.
Genes Clonality established by TCR rearrangements.

Disease Large granular lymphocyte leukemia (LGL) - NK type
Phenotype / cell stem origin TdT-, CD1a
CD2+, CD56+, CD16+, CD7+/-CD3-, CD5-, TCR-Natural killer Activity.
Clinics Aggressive or indolent
Lymphocytosis, splenomegaly, hepatomegaly
Cytogenetics del(6)(q21-25)
Genes TCR chain genes in germ line.

Disease Sezary syndrome (SS)
Phenotype / cell stem origin TdT-, CD1a-, CD3+, CD4+, CD8-, Helper or no functional activity.
Clinics Variable clinical course with skin involvement and cells with cerebriform nuclei.
Cytogenetics Complex, clonal, oligoclonal or nonclonal with variable ploidy.
Genes P53 gene deletion and protein expression in the absence of gene mutation.
Few cases express MDM2

Disease Adult T-cell leukemia lymphoma (ATLL)
Phenotype / cell stem origin TdT-, CD1a-, CD7- CD4+ CD8- CD25+, Suppressor activity.
Clinics Aggressive,
Hypercalcaemia, lymphadenopathy, 'flower cells', HTLV-1 Positive.
Cytogenetics Complex and often oligoclonal.
Numerical abnormalities: 3, 7, X.
Structural abnormalities: 1q, 3q, 6q, 14q.
Genes Oligoclonal/mono clonal integration of HTLV-1in host DNA.
Abnormalities of p53, p16 and p15 genes.

Disease T-NHL hepatosplenic lymphoma
Phenotype / cell stem origin TdT-, CD1a-, CD3+/- CD56+, CD7+, granzyme A+, TCR g/d+
Clinics Aggressive,
Hepato splenomegaly
Cytogenetics Abnormal.7q, i(7q)
Genes TCR genes gamma/delta rearranged but alpha/beta not rearranged.

Disease Peripheral/post-thymic T-cell lymphoma (pleomorphic and immunoblastic subtypes)
Phenotype / cell stem origin TdT-, CD1a-, Variable expression of CD4 or CD8
Clinics Aggressive; advanced stages.
Cytogenetics Variable

Disease Angio immunoblastic T-cell lymphoma
Phenotype / cell stem origin TdT-, CD1a-, CD2+, CD5+, CD3+ CD4+ CD8-
Clinics Disproteinemia, lymphadenopathy, immune abnormalities.
Cytogenetics Complex with multiple related or unrelated clones.
+3 or i(3q), +5, del(6q).
Progression from normal karyotype to abnormal clone observed during transition from hyperplasia to neoplasia.
Genes Integrated EBV sequences present in both B-and T-cells and is unlikely to be the etiological agent.

Disease Angiocentric (nasal) T-cell lymphoma
Phenotype / cell stem origin TdT-, CD1a-, T-cell or NK phenotype.
Clinics Prevalent in Asia and south America; extra nodal involvement.
Cytogenetics i(1q), del(6q), i(6p)
Genes Majority have no TCR rearrangement; EBV clonally integrated and plays a role in the etiology of the disease.

Disease Anaplastic (Ki 1+) large cell lymphoma
Phenotype / cell stem origin TdT-, CD1a-, CD3+/- CD30+ (Ki 1+), CD15-, CD25+, HLA-Dr+, CD71+
Clinics Aggressive with skin nodes and extranodal involvement.
Cytogenetics t(2;5)(p23;q35)
Genes Fusion gene NPM-ALK; 2p23 -Nucleolar phosphoprotein- NPM; 5q35 -Anaplastic lymphoma kinase- ALK.

Disease Intestinal T-cell lymphoma
Phenotype / cell stem origin TdT, CD1a-, CD3+, CD8+, CD103+, CD4-, CD8-
Clinics Bone pain, coeliac disease, mesenteric nodes.
Genes EBV genome present in mexican population but not in the europeans.

Disease T-lymphoblastic Lymphoma/leukaemia (T-Lbly/T-ALL)
Phenotype / cell stem origin TDT+, CD1a+, CD7+, cytCD3+ or +/-, other T-cell antigens.Thymic uncommitted T-cell.
Clinics Aggressive; course similar to ALL.
Mediastinal mass, high WBC.
Cytogenetics del(6)(q21-q22)
t(1;14)(p34;q11); 1p34: tal-1gene; 14q11: TCR alpha
Genes TCR chain genes rearranged.


p53 allele deletion and protein accumulation occurs in the absence of p53 gene mutation in T-prolymphocytic leukaemia and Sezary syndrome.
Brito-Babapulle V, Hamoudi R, Matutes E, Watson S, Kaczmarek P, Maljaie H, Catovsky D
British journal of haematology. 2000 ; 110 (1) : 180-187.
PMID 10930996
Classification of natural killer (NK) cell and NK-like T-cell malignancies.
Jaffe ES
Blood. 1996 ; 87 (4) : 1207-1210.
PMID 8608206
Chromosome abnormalities in adult T-cell leukemia/lymphoma: a karyotype review committee report.
Kamada N, Sakurai M, Miyamoto K, Sanada I, Sadamori N, Fukuhara S, Abe S, Shiraishi Y, Abe T, Kaneko Y
Cancer research. 1992 ; 52 (6) : 1481-1493.
PMID 1540956
Clonal diseases of large granular lymphocytes.
Loughran TP Jr
Blood. 1993 ; 82 (1) : 1-14.
PMID 8324214
CD30-positive large cell lymphomas ('Ki-1 lymphoma') are associated with a chromosomal translocation involving 5q35.
Mason DY, Bastard C, Rimokh R, Dastugue N, Huret JL, Kristoffersson U, Magaud JP, Nezelof C, Tilly H, Vannier JP
British journal of haematology. 1990 ; 74 (2) : 161-168.
PMID 2156548
T-Cell Lymphoproliferative Disorders. Classification, Clinical and Laboratory Aspects.
Matutes E
Advances in Blood Disorders. 2000.
Indication of an involvement of interleukin-1 beta converting enzyme-like protease in intramedullary apoptotic cell death in the bone marrow of patients with myelodysplastic syndromes.
Mundle SD, Venugopal P, Cartlidge JD, Pandav DV, Broady-Robinson L, Gezer S, Robin EL, Rifkin SR, Klein M, Alston DE, Hernandez BM, Rosi D, Alvi S, Shetty VT, Gregory SA, Raza A
Blood. 1996 ; 88 (7) : 2640-2647.
PMID 8839858
Detection of aberrant clones in nearly all cases of angioimmunoblastic lymphadenopathy with dysproteinemia-type T-cell lymphoma by combined interphase and metaphase cytogenetics.
Schlegelberger B, Zhang Y, Weber-Matthiesen K, Grote W
Blood. 1994 ; 84 (8) : 2640-2648.
PMID 7919378
Consistent presence of isochromosome 7q in hepatosplenic T gamma/delta lymphoma: a new cytogenetic-clinicopathologic entity.
Wang CC, Tien HF, Lin MT, Su IJ, Wang CH, Chuang SM, Shen MC, Liu CH
Genes, chromosomes & cancer. 1995 ; 12 (3) : 161-164.
PMID 7536454
Identification of del(6)(q21q25) as a recurring chromosomal abnormality in putative NK cell lymphoma/leukaemia.
Wong KF, Chan JK, Kwong YL
British journal of haematology. 1997 ; 98 (4) : 922-926.
PMID 9326190


This paper should be referenced as such :
Brito-Babapulle, V ; Matutes, E ; Catovsky, D
Classification of T-cell disorders
Atlas Genet Cytogenet Oncol Haematol. 2001;5(2):118-120.
Free journal version : [ pdf ]   [ DOI ]
On line version :

Other genes implicated (Data extracted from papers in the Atlas) [ 10 ]


External links

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