X-linked severe combined immunodeficiency with gamma delta T cells.
Acta Paediatr Jpn
; 39(4): 442-7, 1997 Aug.
Article
in En
| MEDLINE
| ID: mdl-9316288
ABSTRACT
A patient with X-linked severe combined immunodeficiency (X-SCID) was found to have a deletion mutation of a four base pair in the transmembrane domain of the IL-2 receptor gamma chain gene, a subunit shared by the receptors for IL-4, IL-7, IL-9, and IL-15 (common gamma chain; gamma c). He had very few alpha beta T cells but had a considerable number of gamma delta T cells in his peripheral blood. Fluorescence in situ hybridization (FISH) analysis showed that the gamma delta T cells in his peripheral blood were not of maternal origin. He had received a Bacillus Calmette-Guerin (BCG) vaccination before recognition of the disease, and the BCG infection remained quiescent with no reaction for 19 months. After successful bone marrow transplantation, the site of the BCG vaccination showed a reaction, and live BCG were detected. It is useful to consider the relationship between the existence of gamma delta T cells and BCG in this case, and it is suggested that gamma delta T cells may be, in a given situation, less dependent on the gamma c chain than are alpha beta T cells.
Search on Google
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Receptors, Interleukin-2
/
Severe Combined Immunodeficiency
Limits:
Humans
/
Infant
/
Male
Language:
En
Journal:
Acta Paediatr Jpn
Year:
1997
Type:
Article
Affiliation country:
Japan