TCR alpha beta(+)/CD4(+) large granular lymphocytosis - A new clonal T-cell lymphoproliferative disorder

• Type: Article in International Scientific Journal
• Date: 2003

Authors

Lima, M (Author) Other; Almeida, J (Author) Other; Teixeira, MD (Author) Other; Alguero, MD (Author) Other; Santos, AH (Author) Other; Balanzategui, A (Author) Other; Queiros, ML (Author) Other; Barcena, P (Author) Other; Izarra, A (Author) Other; Fonseca, S (Author) Other; Bueno, C (Author) Other; Justica, B (Author) Other; Gonzalez, M (Author) Other; San Miguel, JF (Author) Other; Orfao, A (Author) Other

Journal

Title: American Journal of Pathology

Vol. 163

Pages: 763-771

ISSN: 0002-9440

Publisher: Elsevier

Indexing

ISI Web of Knowledge - 61 Citations

Scopus - 52 Citations

Other information

Authenticus ID: P-000-FKD

DOI: 10.1016/s0002-9440(10)63703-0

Abstract (EN): Large granular lymphocyte (LGL) leukemia is a well-recognized disease of mature T-CD8(+) or less frequently natural killer cells; in contrast, monoclonal expansions of CD4(+) T-LGL have only been sporadically reported in the literature. In the present article we have explored throughout a period of 56 months the incidence of monoclonal expansions of CD4(+) T-LGL in a population of 2.2 million inhabitants and analyzed the immunophenotype and the pattern of cytokine production of clonal CD4(+) T cells of a series of 34 consecutive cases. Like CD8(+) T-LGL leukemias, CD4+ T-LGL leukemia patients have an indolent disease; however, in contrast to CD8(+) T-LGL leukemias, they do not show cytopenias and autoimmune phenomena and they frequently have associated neoplasias, which is usually determining the clinical course of the disease. Monoclonal CD4+ T-LGL showed expression of TCRalphabeta, variable levels of CD8 (CD8(-/+dim)) and a homogeneous typical cytotoxic (granzyme B+, CD56(+), CD57(+), CD11b(+/-)) and activated/memory T cell (CD2(+bright), CD7(-/+dim) CD11a(+bright), CD28(-), CD62L(-) HLA-DR+) immunophenotype. in addition, they exhibited a Th1 pattern of cytokine production [interferon-gamma(++), tumor necrosis factor-alpha(++), interleukin (IL-2)(-/+), IL-4(-), IL-10(-), IL-13(-)]. Phenotypic analysis of the TCR-Vbeta repertoire revealed Large monoclonal TCR-Vbeta expansions; only a restricted number of TCR-Vbeta families were represented in the 34 cases analyzed. These findings suggest that monoclonal TCRalphabeta(+)/CD4(+)/NKa(+)/CD8(-/+dim) T-LGL represent a subgroup of monoclonal LGL lymphoproliferative disorders different from both CD8(+) T-LGL and natural killer cell-type LGL leukemias. Longer follow-up periods are necessary to determine die exact significance of this clonal disorder.

Language: English

Type (Professor's evaluation): Scientific

No. of pages: 9