Abstract
Simple SummaryLarge granular lymphocyte (LGL) leukemia, a lymphoproliferative disease, is characterized by an increased frequency of large-sized lymphocytes with typical expression of T-cell receptor (TCR) αβ, CD3, CD8, CD16, CD45RA, and CD57, and with the expansion of one to three subfamilies of the TCR variable β chain reflecting gene rearrangements. Molecular analysis remains the gold standard for confirmation of TCR clonality; however, flow cytometry is time and labor saving, and can be associated with simultaneous investigation of other surface markers. Moreover, Vβ usage by flow cytometry can be employed for monitoring clonal kinetics during treatment and follow-up of LGL leukemia patients.Large granular lymphocyte (LGL) leukemia is a lymphoproliferative disorder of mature T or NK cells frequently associated with autoimmune disorders and other hematological conditions, such as myelodysplastic syndromes. Immunophenotype of LGL cells is similar to that of effector memory CD8+ T cells with T-cell receptor (TCR) clonality defined by molecular and/or flow cytometric analysis. Vβ usage by flow cytometry can identify clonal TCR rearrangements at the protein level, and is fast, sensitive, and almost always available in every Hematology Center. Moreover, Vβ usage can be associated with immunophenotypic characterization of LGL clone in a multiparametric staining, and clonal kinetics can be easily monitored during treatment and follow-up. Finally, Vβ usage by flow cytometry might identify LGL clones silently underlying other hematological conditions, and routine characterization of Vβ skewing might identify recurrent TCR rearrangements that might trigger aberrant immune responses during hematological or autoimmune conditions.
Highlights
Large granular lymphocyte (LGL) leukemia is a chronic lymphoproliferative disorder arising from clonal expansion of mature T or Natural Killer (NK) cells, and accounts for 2–5% of all cases of non-Hodgkin lymphomas (NHL) in Western countries [1]
In the majority of cases, leukemic cells display a characteristic phenotype with surface expression of T-cell receptor (TCR) αβ, CD3, CD5dim, CD8, CD16, CD45RA, and CD57, while cells are negative for CD4, CD27, CD28, and CD45RO, resembling effector memory and terminal effector memory T cell phenotype [1,7,10]
We focus on biological significance of LGL clonality and clinical utility of TCR repertoire investigation by flow cytometry
Summary
Large granular lymphocyte (LGL) leukemia is a chronic lymphoproliferative disorder arising from clonal expansion of mature T or Natural Killer (NK) cells, and accounts for 2–5% of all cases of non-Hodgkin lymphomas (NHL) in Western countries [1]. LGL leukemia of mature T cells is the most common entity (85% of cases), usually occurring with an indolent clinical course, while. CD4+ CD8+/− T-LGL leukemia has an indolent course and is frequently characterized by lymphocytosis with normal hemoglobin and platelet count and by the presence of a second neoplasms, especially B-cell NHL [24,25]. We focus on biological significance of LGL clonality and clinical utility of TCR repertoire investigation by flow cytometry
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