Angioimmunoblastic T cell lymphoma is derived from mature T-helper cells with varying expression and loss of detectable CD4.
Int J Cancer
Angioimmunoblastic T cell lymphoma (AILT) is a rare lymphoma that is regarded as a clinicopathologic entity but shows considerable histomorphologic diversity, variable immunophenotypes and inconsistent T cell receptor (TCR) gene rearrangement. One hundred four paraffin blocks of AILT were investigated defining tumor cell lineage by triple immunostains with a confocal laser scanning microscope and correlating morphology, immunophenotype and TCRgamma gene rearrangement to clinical outcome. Ninety-nine cases were CD4(+), some of them showing a mixture of CD4(+) and CD4(-) tumor cells. The remaining 5 specimens were CD3(+)/CD4(-)/CD8(-). A considerable number of T cells of different subtypes could always be found, but even in 13 cases predominated by CD8(+) cells, proliferation could be attributed to atypical CD4(+) cells. TCRgamma gene rearrangement was monoclonal in 48 cases (69%) among 70 tested. In 29 of these semi-quantitative gene scan analysis resulted in a median proportion of monoclonal peak of 35% of PCR-products. Clinical outcome was identical grouping patients by clonality of TCRgamma, absence or presence of clear cell clusters and international prognostic index. We conclude that AILT is mainly derived from CD2(+)CD3(+)CD4(+)CD5(+)CD7(-) mature T-helper cells with varying expression and partial loss of detectable CD4. A significant number of non-neoplastic T cells (resting CD4(+) T cells and activated small or medium-sized CD8(+) lymphocytes) may coexist with a minor neoplastic T cell population. Clinicopathologic correlation suggests AILT to be a well defined homogeneous entity with poor prognosis. Currently no prognostic factors can be derived.
|Title:||Angioimmunoblastic T cell lymphoma is derived from mature T-helper cells with varying expression and loss of detectable CD4.|
|Keywords:||Adult, Aged, Aged, 80 and over, Antigens, CD4, Antigens, Differentiation, T-Lymphocyte, CD4-CD8 Ratio, CD4-Positive T-Lymphocytes, Cell Lineage, Female, Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor, Humans, Immunoblastic Lymphadenopathy, Immunoenzyme Techniques, Immunophenotyping, L-Lactate Dehydrogenase, Lymph Nodes, Lymphoma, T-Cell, Male, Microscopy, Confocal, Middle Aged, Polymerase Chain Reaction, T-Lymphocytes, Helper-Inducer|
|UCL classification:||UCL > School of Life and Medical Sciences
UCL > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science
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