T-lymphoblastic lymphomas expressing the non-disulfide-linked form of the T-cell receptor γ/δ: Characterization with monoclonal antibodies and genotypic analysis

B. Falini, L. Flenghi, M. Fagioli, M. F. Martelli, S. Pileri, F. Grignani, A. Beltrami, D. Novero, P. G. Pelicci

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Abstract

Three cases of T-lymphoblastic lymphomas (T-LL) expressing the T cell antigen receptor γδ (TCRγδ) are reported. All of them were CD3+/βF1-/TCRδ1+. Moreover, neoplastic cells reacted with the δTCS1 monoclonal antibody (MoAb) which binds to the non-disulfide-linked form of the TCRγδ, but not with the BB3 MoAb which recognizes the disulfide-linked form of the TCRγδ. All cases showed a stage II cortical phenotype, eg, TdT+/CD1+/CD3+/CD5+/CD7+; two of them coexpressed CD4/CD8, while the other was CD4+/CD8-. Two cases were positive for CALLA and CD25. Immunogenotypic analysis showed evidence of Tβ and Cγ2 gene rearrangements in all three cases and immunoglobulin (Ig) gene rearrangements in two cases. Two patients presented with an anterior mediastinal mass and the third with a solitary inguinal lymphadenopathy. We suggest that these cases of TCRγδ+ T-LL may be derived from the small population (approximately 0.5%) of CD3+ cortical thymocytes which, in the normal human thymus, express the δTCS1-reactive, non-disulfide-linked form of the TCRγδ.

Original languageEnglish
Pages (from-to)2501-2507
Number of pages7
JournalBlood
Volume74
Issue number7
Publication statusPublished - 1989

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T-Cell Antigen Receptor
Precursor Cell Lymphoblastic Leukemia-Lymphoma
Monoclonal Antibodies
Gene Rearrangement
Genes
Thymus
Immunoglobulin Genes
Groin
Thymocytes
Disulfides
Thymus Gland
Immunoglobulins
Phenotype
Population

ASJC Scopus subject areas

  • Hematology

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T-lymphoblastic lymphomas expressing the non-disulfide-linked form of the T-cell receptor γ/δ : Characterization with monoclonal antibodies and genotypic analysis. / Falini, B.; Flenghi, L.; Fagioli, M.; Martelli, M. F.; Pileri, S.; Grignani, F.; Beltrami, A.; Novero, D.; Pelicci, P. G.

In: Blood, Vol. 74, No. 7, 1989, p. 2501-2507.

Research output: Contribution to journalArticle

Falini, B. ; Flenghi, L. ; Fagioli, M. ; Martelli, M. F. ; Pileri, S. ; Grignani, F. ; Beltrami, A. ; Novero, D. ; Pelicci, P. G. / T-lymphoblastic lymphomas expressing the non-disulfide-linked form of the T-cell receptor γ/δ : Characterization with monoclonal antibodies and genotypic analysis. In: Blood. 1989 ; Vol. 74, No. 7. pp. 2501-2507.
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abstract = "Three cases of T-lymphoblastic lymphomas (T-LL) expressing the T cell antigen receptor γδ (TCRγδ) are reported. All of them were CD3+/βF1-/TCRδ1+. Moreover, neoplastic cells reacted with the δTCS1 monoclonal antibody (MoAb) which binds to the non-disulfide-linked form of the TCRγδ, but not with the BB3 MoAb which recognizes the disulfide-linked form of the TCRγδ. All cases showed a stage II cortical phenotype, eg, TdT+/CD1+/CD3+/CD5+/CD7+; two of them coexpressed CD4/CD8, while the other was CD4+/CD8-. Two cases were positive for CALLA and CD25. Immunogenotypic analysis showed evidence of Tβ and Cγ2 gene rearrangements in all three cases and immunoglobulin (Ig) gene rearrangements in two cases. Two patients presented with an anterior mediastinal mass and the third with a solitary inguinal lymphadenopathy. We suggest that these cases of TCRγδ+ T-LL may be derived from the small population (approximately 0.5{\%}) of CD3+ cortical thymocytes which, in the normal human thymus, express the δTCS1-reactive, non-disulfide-linked form of the TCRγδ.",
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T2 - Characterization with monoclonal antibodies and genotypic analysis

AU - Falini, B.

AU - Flenghi, L.

AU - Fagioli, M.

AU - Martelli, M. F.

AU - Pileri, S.

AU - Grignani, F.

AU - Beltrami, A.

AU - Novero, D.

AU - Pelicci, P. G.

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AB - Three cases of T-lymphoblastic lymphomas (T-LL) expressing the T cell antigen receptor γδ (TCRγδ) are reported. All of them were CD3+/βF1-/TCRδ1+. Moreover, neoplastic cells reacted with the δTCS1 monoclonal antibody (MoAb) which binds to the non-disulfide-linked form of the TCRγδ, but not with the BB3 MoAb which recognizes the disulfide-linked form of the TCRγδ. All cases showed a stage II cortical phenotype, eg, TdT+/CD1+/CD3+/CD5+/CD7+; two of them coexpressed CD4/CD8, while the other was CD4+/CD8-. Two cases were positive for CALLA and CD25. Immunogenotypic analysis showed evidence of Tβ and Cγ2 gene rearrangements in all three cases and immunoglobulin (Ig) gene rearrangements in two cases. Two patients presented with an anterior mediastinal mass and the third with a solitary inguinal lymphadenopathy. We suggest that these cases of TCRγδ+ T-LL may be derived from the small population (approximately 0.5%) of CD3+ cortical thymocytes which, in the normal human thymus, express the δTCS1-reactive, non-disulfide-linked form of the TCRγδ.

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