Novel primary thymic defect with T lymphocytes expressing gamma delta T cell receptor
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Novel primary thymic defect with T lymphocytes expressing gamma delta T cell receptor. / Geisler, C; Pallesen, G; Platz, P; Odum, N; Dickmeiss, E; Ryder, L P; Svejgaard, A; Plesner, T; Larsen, J K; Koch, C.
In: Journal of Clinical Pathology, Vol. 42, No. 7, 1989, p. 705-11.Research output: Contribution to journal › Journal article › Research › peer-review
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TY - JOUR
T1 - Novel primary thymic defect with T lymphocytes expressing gamma delta T cell receptor
AU - Geisler, C
AU - Pallesen, G
AU - Platz, P
AU - Odum, N
AU - Dickmeiss, E
AU - Ryder, L P
AU - Svejgaard, A
AU - Plesner, T
AU - Larsen, J K
AU - Koch, C
N1 - Keywords: Antigens, CD3; Antigens, Differentiation, T-Lymphocyte; Child; Female; Flow Cytometry; Gene Rearrangement, T-Lymphocyte; Humans; Immunologic Deficiency Syndromes; Membrane Glycoproteins; Receptors, Antigen, T-Cell; T-Lymphocytes; Thymus Gland
PY - 1989
Y1 - 1989
N2 - 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.
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.
M3 - Journal article
C2 - 2527256
VL - 42
SP - 705
EP - 711
JO - Journal of Clinical Pathology
JF - Journal of Clinical Pathology
SN - 0021-9746
IS - 7
ER -
ID: 8546484