Hypogammaglobulinemia with impaired T-cell function: suspected maturational defect from CD4+CD45R+ to CD4+CD45R- phenotype.:suspected maturational defect from CD4+CD45R+ to CD4+CD45R- phenotype
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概要
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The author reported a case of 18-month-old girl with hypogammaglobulinemia. She had recurrent upper respiratory tract infections since 6 month old. Serum immunoglobulin values were IgG 311 mg/dl, IgM 15 mg/dl and IgA 45 mg/dl respectively. Antibodies against varicella and polio were negative despite vaccination, but IgM antibody against pertussis was positive after vaccination. Immunophenotypical analysis of PBL revealed normal numbers of CD20+, CD8+, CD4+ and CD16+ cells, but increased ratio of CD4+CD45R+/ CD4+ cells. In vitro, B cells proliferated well in response to SAC, T-cell independent B cell mitogen, and differentiated to secrete enough amount of IgG, IgM and IgA in combination of SAC and IL2, indicating that B sells were maturated enough to respond to cytokines with BCDF activity and secrete Ig. In PWM-driven Ig synthesis, patient PBL secreted low to moderate amount of IgM and very low amount of IgG and IgA. In co-culture assay, patient T-cells did not help control nT to secrete Ig, but control T partially helped patient nT to secrete Ig. Proliferative response of patient T to PHA was decreased and recovered by exogeneous IL2 completely, indicating impaired production of IL2. In vitro change of CD45R expression on CD4+ T-cells in response to PHA plus IL2 was also impaired compared to control. These results may suggest that there is a maturational defect or delay in patient's T-cells from CD4+CD45R+ to CD4+ CD45R- phenotype, and this defect could be responsible for patient T-cell's disability to help Ig production. This impaired T-cell function might be transient or not is remained elucidated, but this case is unique, and helpful for understanding of mechanism of hypogammaglobulinemia which might be due to T-cell dysfunction.
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