Angioimmunoblastic T-cell lymphoma

2002-06-01   Gianluigi Castoldi , Antonio Cuneo 

1.Hematology Section, Department of Biomedical Sciences, University of Ferrara, Corso Giovecca 203, Ferrara, Italy

Clinics and Pathology

Phenotype stem cell 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.

Cytogenetics

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.

    Bibliography

    Pubmed IDLast YearTitleAuthors
    26425711989Angioimmunoblastic lymphadenopathy and related disorders: a retrospective look in search of definitions.Frizzera G et al
    90864421997Clonal identification of trisomies 3, 5 and X in angioimmunoblastic lymphadenopathy with dysproteinemia by fluorescence in situ hybridization.Kumaravel TS et al
    86367761996Significance of cytogenetic findings for the clinical outcome in patients with T-cell lymphoma of angioimmunoblastic lymphadenopathy type.Schlegelberger B et al

    Citation

    Gianluigi Castoldi ; Antonio Cuneo

    Angioimmunoblastic T-cell lymphoma

    Atlas Genet Cytogenet Oncol Haematol. 2002-06-01

    Online version: http://atlasgeneticsoncology.org/haematological/2124/angioimmunoblastic-t-cell-lymphoma

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