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(American Journal of Pathology. 2003;163:763-771.)
© 2003 American Society for Investigative Pathology

TCR{alpha}ß+/CD4+ Large Granular Lymphocytosis

A New Clonal T-Cell Lymphoproliferative Disorder

Margarida Lima*, Julia Almeida{dagger}{ddagger}, Maria dos Anjos Teixeira*, Maria del Carmen Alguero{dagger}, Ana Helena Santos*, Ana Balanzategui{ddagger}§, Maria Luís Queirós*, Paloma Bárcena{dagger}{ddagger}, Antonio Izarra§, Sónia Fonseca*, Clara Bueno{dagger}{ddagger}, Benvindo Justiça*, Marcos Gonzalez{ddagger}§, Jesús F. San Miguel{ddagger}§ and Alberto Orfao{dagger}{ddagger}

From the Serviço de Hematologia Clinica,* Unidade de Citometria, Hospital Geral de Santo Antonio, Porto, Portugal; the Servicio de Citometria,{dagger} Universidad de Salamanca, Salamanca, Spain; the Centro de Investigación del Cáncer,{ddagger} Salamanca, Spain; and the Servicio de Hematología,§ Hospital Universitario de Salamanca, Salamanca, Spain

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-LGLshowed expression of TCR{alpha}ß, 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-Vß repertoire revealed large monoclonal TCR-Vß expansions; only a restricted number of TCR-Vß families were represented in the 34 cases analyzed. These findings suggest that monoclonal TCR{alpha}ß+/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 the exact significance of this clonal disorder.




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