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Angioimmunoblastic T-cell lymphoma

Written2002-06Antonio Cuneo, Gianluigi Castoldi
Hematology Section, Department of Biomedical Sciences, University of Ferrara, Corso Giovecca 203, Ferrara, Italy

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ICD-Morpho 9738/3 Large B-cell lymphoma arising in HHV8-associated multicentric Castleman disease
ICD-Morpho 9705/3 Angioimmunoblastic T-cell lymphoma
Atlas_Id 2124
Other namesAngioimmunoblastic lymphadenopathy with disprotidemia

Clinics and Pathology

Phenotype / cell stem origin The lymphoma cell is a peripheral T lymphocyte in various stages of differentiation. The neoplastic clone expresses T-cell antigens and is usually CD4+. The malignant T-cells are believed to secrete cytokines responsible for the polyclonal B-cell hyperplasia observed in involved nodes. Clonality studies demonstrated a monoclonal rearrangement of the b-chain of the T-cell receptor (TCR) in the majority of cases. In some cases clonality could not be demonstrated. This led some authors to postulate the existence of at least two types of AILD, namely a reactive and benign type and a lymphomatous form.
Etiology The disease is rare
Clinics The disease preferentially affects elderly males (male-to-female ratio 3:1, median age around 60 years). Most patients present with generalized lymphadenopathy, hepatosplenomegaly, skin rash and general symptoms (fever, weight loss). Polyclonal hypergammaglobulinemia is a common finding.
Pathology The lymph node architecture is effaced and no reactive germinal centres are usually observed. The infiltrate may involve the perinodal fat. There is a proliferation of high endothelial venules with clusters of follicular dendritic cells. The lymphoid infiltrate consists of small-to-large cells resembling immunoblasts and atypical clear cells with round nucleus and abundant pale cytoplasm. The latter cells may occur in small aggregates or sheets.
Treatment Some patients respond to steroids; in steroid-unresponsive patients multiagent chemotherapy usually produces short lasting responses.
Evolution Few patients present spontaneous or steroid-induced remission; the majority of cases feature an aggressive disease with short survival despite chemotherapy. Most patients die with infection and active disease.
Prognosis Median survival is about 1-3 years.


Note A mixture of normal and abnormal cells is usually seen in the vast majority of cases. The cytogenetic picture at disease presentation may be normal in some cases which may develop clonal abnormalites during the course of the disease. The following karyotype pattern can be found
Cytogenetics Morphological
  • Clonal abnormalities defining a stemline, with one or more sidelines (approximately 30-50% of the cases)
  • Normal karyotype (10-30% of the cases)
  • Single cells with unrelated chromosome anomalies (10-20%)
  • Unrelated clones with aberrant karyotypes, each carrying single unrelated additional anomalies (10-20%).
  • Recurrent chromosome changes in those cases with an abnormal clone include trisomy 3, trisomy 5 and trisomy X A 14q+ chromosome is a recurrent structural defect. Recurrent breakpoints include 1p31-32; 3p24-25; 4p13; 9q21-22; 12q13; 14q11; 14q32
  • The presence of abnormal metapahses in unstimulated cultures was associated with failure to respond to therapy and with shorter survival, as was the case with +X, structural aberrations of chromosome 1, and complex karyotype. The latter cytogenetic parameter maintained prognostic predictivity at multivariate analysis.
  • Cytogenetics Molecular Using probes for the detection of +3, +5 and +X, the vast majority of cases can be shown to carry aneuploidy.


    Other peripheral T-cell lymphomas.
    Delmer A, Zittoun R
    Magrath I (Ed) The non Hodgkin's lymphomas 2nd edition..
    Angioimmunoblastic lymphadenopathy and related disorders: a retrospective look in search of definitions.
    Frizzera G, Kaneko Y, Sakurai M
    Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K. 1989 ; 3 (1) : 1-5.
    PMID 2642571
    Clonal identification of trisomies 3, 5 and X in angioimmunoblastic lymphadenopathy with dysproteinemia by fluorescence in situ hybridization.
    Kumaravel TS, Tanaka K, Arif M, Ohshima K, Ohgami A, Takeshita M, Kikuchi M, Kamada N
    Leukemia & lymphoma. 1997 ; 24 (5-6) : 523-532.
    PMID 9086442
    Significance of cytogenetic findings for the clinical outcome in patients with T-cell lymphoma of angioimmunoblastic lymphadenopathy type.
    Schlegelberger B, Zwingers T, Hohenadel K, Henne-Bruns D, Schmitz N, Haferlach T, Tirier C, Bartels H, Sonnen R, Kuse R
    Journal of clinical oncology : official journal of the American Society of Clinical Oncology. 1996 ; 14 (2) : 593-599.
    PMID 8636776
    Cytogenetics of non Hodgkin's lymphomas
    WhangPeng J, Knutsen T
    Magrath I (Ed) The non Hodgkin's lymphomas 2nd edition..


    This paper should be referenced as such :
    Cuneo, A ; Castoldi, GL
    Angioimmunoblastic T-cell lymphoma
    Atlas Genet Cytogenet Oncol Haematol. 2002;6(4):288-289.
    Free journal version : [ pdf ]   [ DOI ]
    On line version :

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


    External links

    COSMICHisto = - Site = haematopoietic_and_lymphoid_tissue (COSMIC)
    arrayMap (UZH-SIB Zurich)Topo ( C42) Morph ( 9738/3) -   [auto + random 100 samples .. if exist ]   [tabulated segments]
    arrayMap (UZH-SIB Zurich)Topo ( C42) Morph ( 9705/3) -   [auto + random 100 samples .. if exist ]   [tabulated segments]
    REVIEW articlesautomatic search in PubMed
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