TCR gamma delta(+) large granular lymphocyte leukemias reflect the spectrum of normal antigen-selected TCR gamma delta(+) T-cells

• Type: Article in International Scientific Journal
• Date: 2006

Authors

Sandberg, Y (Author) Other; Almeida, J (Author) Other; Gonzalez, M (Author) Other; Lima, M (Author) Other; Bacrcena, P (Author) Other; Szczepanski, T (Author) Other; van Gastel Mol, EJ (Author) Other; Wind, H (Author) Other; Balanzategui, A (Author) Other; van Dongen, JJM (Author) Other; San Miguel, JF (Author) Other; Orfao, A (Author) Other; Langerak, A (Author) Other

Journal

Title: Leukemia

Vol. 20

Pages: 505-513

ISSN: 0887-6924

Publisher: Springer Nature

Indexing

ISI Web of Knowledge - 43 Citations

Scopus - 33 Citations

Other information

Authenticus ID: P-004-N2P

DOI: 10.1038/sj.leu.2404112

Abstract (EN): T-cell large granular lymphocytes (LGL) proliferations range from reactive expansions of activated T cells to T-cell leukemias and show variable clinical presentation and disease course. The vast majority of T-LGL proliferations express TCR alpha beta. Much less is known about the characteristics and pathogenesis of TCRcd gamma delta(+) cases. We evaluated 44 patients with clonal TCR gamma delta(+) T-LGL proliferations with respect to clinical data, immunophenotype and TCR gene rearrangement pattern. TCR gamma delta(+) T-LGL leukemia patients had similar clinical presentations as TCR alpha beta(+) T-LGL leukemia patients. Their course was indolent and 61% of patients were symptomatic. The most common clinical manifestations were chronic cytopenias - neutropenia (48%), anemia (23%), thrombocytopenia (9%), pancytopenia (2%) and to a lesser extent splenomegaly (18%). Also multiple associated autoimmune (34%) and hematological (14%) disorders were found. Leukemic LGLs were predominantly positive for CD2, CD5, CD7, CD8, and CD57, whereas variable expression was seen for CD16, CD56, CD11b, and CD11c. The Vc9/Vd2 immunophenotype was found in 48% of cases and 43% of cases was positive for V delta 1, reflecting the TCR-spectrum of normal TCR gamma delta(+) T-cells in adult PB. Identification of the well-defined post-thymic V delta 2-J delta 1 selection determinant in all evaluable V gamma 9(+)N delta 2(+) patients, is suggestive of common (super) antigen involvement in the pathogenesis of these TCR gamma delta(+) T-LGL leukemia patients.

Language: English

Type (Professor's evaluation): Scientific

No. of pages: 9