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The Value of Flow Cytometry Clonality in Large Granular Lymphocyte Leukemia
Valentina Giudice1,2, Matteo D'Addona1,2, Nunzia Montuori3
1Department of Medicine, Surgery, and Dentistry, Scuola Medica Salernitana, University of Salerno, 84081 Baronissi, Italy.
Insights
Flow cytometry Vβ usage effectively identifies clonal T-cell receptor rearrangements in large granular lymphocyte leukemia. This method aids in diagnosing LGL leukemia and monitoring its progression, even in silent underlying hematological conditions.
Area of Science:
- Hematology
- Immunology
- Oncology
Background:
- Large granular lymphocyte (LGL) leukemia is a T or NK cell lymphoproliferative disorder.
- It often co-occurs with autoimmune diseases and myelodysplastic syndromes.
- LGL immunophenotype resembles effector memory CD8+ T cells.
Purpose of the Study:
- To evaluate flow cytometry Vβ usage for identifying T-cell receptor (TCR) clonality in LGL leukemia.
- To assess the utility of Vβ usage in characterizing LGL clones and monitoring disease kinetics.
- To explore Vβ usage for detecting silent LGL clones in other hematological conditions.
Main Methods:
- Flow cytometry analysis of Vβ usage to detect clonal TCR rearrangements at the protein level.
- Multiparametric staining for immunophenotypic characterization of LGL clones.
- Monitoring clonal kinetics during treatment and follow-up.
Main Results:
- Vβ usage by flow cytometry is a fast, sensitive method for identifying clonal TCR rearrangements.
- This technique allows for immunophenotypic characterization and monitoring of LGL clones.
- Vβ skewing can reveal recurrent TCR rearrangements linked to aberrant immune responses.
Conclusions:
- Flow cytometry Vβ usage is a valuable tool for diagnosing and managing LGL leukemia.
- It can identify LGL clones underlying other hematological conditions.
- Routine Vβ analysis may aid in understanding immune dysregulation in hematological and autoimmune disorders.
Abstract:
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.
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