Abstract
We describe here one 8-year-old girl with an unusual form of immunodeficiency, characterized by hypogammaglobulinemia with hyper-IgM, severe T-cell defect, and chronic lymphadenopathy. Patient's B cells failed to produce IgG or IgA in vitro following stimulation with either pokeweed mitogen or Epstein-Barr virus, suggesting an intrinsic B-cell defect. Abnormal T-cell function was demonstrated by impaired in vivo delayed type hypersensitivity, reduction of mitogen-induced proliferation and interleukin 2 production, reduction of interferon-γ production, and marked decrease of circulating OKT4+ cells. The latter cells were found in normal proportion in the patient's lymph node tissue. This finding suggests that the decrease of OKT4+ cells in peripheral blood was due to the abnormal recirculation of these cells. The identity of this syndrome with the infantile form of the acquired immunodeficiency syndrome was apparently ruled out by the failure to demonstrate HTLV-III-related sequences in patient's lymphocytes or virus-specific serum antibodies.
| Original language | English |
|---|---|
| Pages (from-to) | 256-264 |
| Number of pages | 9 |
| Journal | Clinical Immunology and Immunopathology |
| Volume | 38 |
| Issue number | 2 |
| DOIs | |
| Publication status | Published - 1986 |
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ASJC Scopus subject areas
- Immunology
- Immunology and Allergy
- Pathology and Forensic Medicine
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Hypogammaglobulinemia with hyper-IgM, severe T-cell defect, and abnormal recirculation of OKT4 lymphocytes in a girl with chronic lymphadenopathy. / Fiorilli, Massimo; Russo, Giandomenico; Paganelli, Roberto; Papetti, Claudia; Carbonari, Maurizio; Crescenzi, Marco; Calvani, Mario; Quinti, Isabella; Aiuti, Fernando.
In: Clinical Immunology and Immunopathology, Vol. 38, No. 2, 1986, p. 256-264.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Hypogammaglobulinemia with hyper-IgM, severe T-cell defect, and abnormal recirculation of OKT4 lymphocytes in a girl with chronic lymphadenopathy
AU - Fiorilli, Massimo
AU - Russo, Giandomenico
AU - Paganelli, Roberto
AU - Papetti, Claudia
AU - Carbonari, Maurizio
AU - Crescenzi, Marco
AU - Calvani, Mario
AU - Quinti, Isabella
AU - Aiuti, Fernando
PY - 1986
Y1 - 1986
N2 - We describe here one 8-year-old girl with an unusual form of immunodeficiency, characterized by hypogammaglobulinemia with hyper-IgM, severe T-cell defect, and chronic lymphadenopathy. Patient's B cells failed to produce IgG or IgA in vitro following stimulation with either pokeweed mitogen or Epstein-Barr virus, suggesting an intrinsic B-cell defect. Abnormal T-cell function was demonstrated by impaired in vivo delayed type hypersensitivity, reduction of mitogen-induced proliferation and interleukin 2 production, reduction of interferon-γ production, and marked decrease of circulating OKT4+ cells. The latter cells were found in normal proportion in the patient's lymph node tissue. This finding suggests that the decrease of OKT4+ cells in peripheral blood was due to the abnormal recirculation of these cells. The identity of this syndrome with the infantile form of the acquired immunodeficiency syndrome was apparently ruled out by the failure to demonstrate HTLV-III-related sequences in patient's lymphocytes or virus-specific serum antibodies.
AB - We describe here one 8-year-old girl with an unusual form of immunodeficiency, characterized by hypogammaglobulinemia with hyper-IgM, severe T-cell defect, and chronic lymphadenopathy. Patient's B cells failed to produce IgG or IgA in vitro following stimulation with either pokeweed mitogen or Epstein-Barr virus, suggesting an intrinsic B-cell defect. Abnormal T-cell function was demonstrated by impaired in vivo delayed type hypersensitivity, reduction of mitogen-induced proliferation and interleukin 2 production, reduction of interferon-γ production, and marked decrease of circulating OKT4+ cells. The latter cells were found in normal proportion in the patient's lymph node tissue. This finding suggests that the decrease of OKT4+ cells in peripheral blood was due to the abnormal recirculation of these cells. The identity of this syndrome with the infantile form of the acquired immunodeficiency syndrome was apparently ruled out by the failure to demonstrate HTLV-III-related sequences in patient's lymphocytes or virus-specific serum antibodies.
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UR - http://www.scopus.com/inward/citedby.url?scp=0022643294&partnerID=8YFLogxK
U2 - 10.1016/0090-1229(86)90143-1
DO - 10.1016/0090-1229(86)90143-1
M3 - Article
C2 - 3079686
AN - SCOPUS:0022643294
VL - 38
SP - 256
EP - 264
JO - Clinical Immunology and Immunopathology
JF - Clinical Immunology and Immunopathology
SN - 0090-1229
IS - 2
ER -