Clonality of CD3 negative large granular lymphocyte proliferations determined by PCR based X-inactivation studies.

Abstract

AIMS:

To examine persistent CD3-large granular lymphocytosis (LGL) cases for clonality, both by lineage specific (T cell receptor) and lineage independent (X-inactivation) molecular methods; and to find out whether X-inactivation studies are more appropriate than gene rearrangement studies for this subset of LGL disorders.

METHODS:

Patients were selected who had LGL of more than six months' duration and identified as CD3- by immunophenotyping. T cell receptor studies and, where possible, X-inactivation studies of the phosphoglycerate kinase (PGK) gene were carried out. Analysis of subpopulations was carried out on cases heterozygous for PGK by the use of a polymerase chain reaction (PCR) method for X-inactivation.

RESULTS:

Of 17 CD3- LGL cases studied, all were found to be germline for beta, gamma, and delta T cell receptor studies, and immunoglobulin heavy chain genes. However, six of these were analysed by X-inactivation of the PGK gene and two cases gave clonal band patterns but only within the CD3- subpopulation.

CONCLUSIONS:

Clonal analysis by the lineage independent method of X-inactivation allows clonal expansion undetected by T and B cell specific markers to be identified. It is therefore a more appropriate method for the analysis of CD3- LGL. This has implications for diagnosis in CD3- LGL disorders.

• Authors: A Kelly, S J Richards, M Sivakumaran, C Shiach, A D Stewart, B E Roberts, C S Scott
• Journal: Journal of clinical pathology (J Clin Pathol) Vol. 47 Issue 5 Pg. 399-404 (May 1994) ISSN: 0021-9746 [Print] England
• PMID: 8027391 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• CD3 Complex
• Immunoglobulin Heavy Chains
• Phosphoglycerate Kinase

Topics

• Adult
• Aged
• Base Sequence
• CD3 Complex (blood)
• Clone Cells (immunology)
• Dosage Compensation, Genetic
• Female
• Gene Rearrangement
• Gene Rearrangement, T-Lymphocyte
• Humans
• Immunoglobulin Heavy Chains (genetics)
• Immunomagnetic Separation
• Immunophenotyping (methods)
• Killer Cells, Natural (immunology)
• Lymphocytosis (enzymology, genetics, immunology)
• Middle Aged
• Molecular Sequence Data
• Phosphoglycerate Kinase (genetics)
• Polymerase Chain Reaction