RT Journal Article
SR Electronic
T1 Novel primary thymic defect with T lymphocytes expressing gamma delta T cell receptor.
JF Journal of Clinical Pathology
JO J Clin Pathol
FD BMJ Publishing Group Ltd and Association of Clinical Pathologists
SP 705
OP 711
DO 10.1136/jcp.42.7.705
VO 42
IS 7
A1 Geisler, C
A1 Pallesen, G
A1 Platz, P
A1 Odum, N
A1 Dickmeiss, E
A1 Ryder, L P
A1 Svejgaard, A
A1 Plesner, T
A1 Larsen, J K
A1 Koch, C
YR 1989
UL http://jcp.bmj.com/content/42/7/705.abstract
AB Flow cytometric analysis of the peripheral blood mononuclear cells in a six year old girl with a primary cellular immune deficiency showed a normal fraction of CD3 positive T cells. Most (70%) of the CD3 positive cells, however, expressed the gamma delta and not the alpha beta T cell receptor. Immunoprecipitation and sodium dodecyl sulphate-polyacrylamide gel electrophoresis (SDS-PAGE) showed that most of the gamma delta T cell receptors existed as disulphide-linked heterodimers. Proliferative responses to mitogens were severely reduced, but specific antibody responses after vaccination could be detected. A thymic biopsy specimen showed severe abnormalities of both the thymic lymphoid and epithelial component with abortive medullary differentiation and almost an entire lack of Hassall's corpuscles. This patient represents a case of primary immune deficiency syndrome not previously described. Thymic deficiency associated with a high proportion of T cells expressing the gamma delta T cell receptor has been described in nude mice, and it is suggested that the immune deficiency of this patient may represent a human analogue.