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Aggressive acute CD3+, CD56- T cell large granular lymphocyte leukemia with two stages of maturation arrest
R Tordjman1, E Macintyre, J F Emile
1Department of Clinical and Biological Hematology, Hopital Necker, Paris, France.
Insights
This study details an unusual T-large granular lymphocyte (LGL) leukemia case presenting acutely with unique cell surface markers and maturation arrest. Findings suggest a potential T-cell maturation process or selective CD8+ cell circulation in T-LGL leukemia.
Area of Science:
- Hematology
- Immunology
- Oncology
Background:
- T-large granular lymphocyte (LGL) leukemia is typically a chronic condition characterized by specific immunophenotypes (CD3+, CD8+, CD16+, CD57+, CD56-).
- Common clinical manifestations include neutropenia, rheumatoid arthritis, and splenomegaly.
Observation:
- An unusual T-LGL leukemia case presented with acute features, large tumor mass, and high LGL counts.
- The leukemic cells exhibited an atypical phenotype: CD3cyt+, CD3surface-, CD16+, CD56-.
- Two distinct maturation arrest stages were observed: CD4+, CD8+ cells in lymph nodes and predominantly CD8+ cells in circulation.
Findings:
- T-cell receptor gamma (TCRγ) gene analysis confirmed a single T-cell clone origin for both cell populations.
- The findings suggest a potential maturation process between CD4+/CD8+ and CD8+ T-LGL populations or preferential circulation of CD8+ cells.
Implications:
- This case expands the understanding of T-LGL leukemia heterogeneity and presentation.
- It highlights the importance of detailed immunophenotyping and molecular analysis in diagnosing atypical LGL leukemia cases.
- The study provides insights into T-cell maturation pathways and clonal evolution in leukemia.
Abstract:
In the majority of clonal expansions of CD3+ large granular lymphocytes (LGL), referred to as T-LGL leukemia, patients have a chronic disease, often manifested by severe neutropenia, rheumatoid arthritis, and mild to moderate splenomegaly. The characteristic leukemic phenotype is CD3+, CD8+, CD16+, CD57+ and CD56-. Here we report an unusual case of T-LGL (CD3cyt+, CD3surface-, CD16+, CD56-) with clinicopathological features (acute presentation, large tumor mass, and systemic illness with highLGL counts at diagnosis) similar to those described for patients with CD3-natural killer (NK)-LGL leukemia. Two distinct stages of maturation arrest were observed: in the lymph node abnormal cells were CD4+, CD8+ whereas the majority of circulating leukemic cells expressed only CD8. TCR gamma (TCR gamma) gene configuration demonstrated that these originated from the same T cell clone, suggesting a maturation process between the two populations, or preferential passage of CD8 single positive cells into the blood.