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Chromosomal abnormalities define clonal proliferation in CD3- large granular lymphocyte leukemia
M Taniwaki1, S Tagawa, H Nishigaki
1Department of Medicine, Kyoto Prefectural University of Medicine, Japan.
American Journal of Hematology
|January 1, 1990
Summary
Chromosomal abnormalities indicate clonal proliferation in large granular lymphocyte (LGL) disorders. CD3- LGL patients with these abnormalities exhibited aggressive disease, confirming clonal origin in aggressive LGL leukemia.
Area of Science:
- Hematology
- Oncology
- Cytogenetics
Background:
- Large granular lymphocyte (LDGL) disorders can present with varying clinical outcomes.
- The T cell lineage antigen CD3 expression is a potential marker for disease behavior.
Purpose of the Study:
- To investigate chromosomal abnormalities in patients with lymphoproliferative disorder of large granular lymphocytes (LDGL).
- To correlate chromosomal abnormalities with clinical presentation and T cell phenotype (CD3 expression).
Main Methods:
- Karyotyping of lymphocytes from six LDGL patients.
- Analysis of CD3 antigen expression on T cells.
- Assessment of T cell receptor beta-chain gene configuration.
Main Results:
- Chromosomal abnormalities were detected in four of six LDGL patients.
- Three of four CD3- LDGL patients with abnormalities showed aggressive disease, including hepatosplenomegaly.
- All three CD3- LDGL patients with chromosomal abnormalities had a germ-line configuration of the T cell receptor beta-chain gene.
Conclusions:
- Chromosomal abnormalities provide definitive evidence for clonal origin in expanded LDGL cells.
- The CD3- phenotype in LDGL is associated with chromosomal abnormalities and aggressive disease, suggesting a distinct pathogenetic mechanism.