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RATIONALE: Our patient with Good's syndrome suffered from recurrent CMV infection despite adequate IVIg replacement. To assess for possible T cell defect in this patient, CMV-specific T cells number and function was evaluated. METHODS: Peripheral blood T cells were evaluated at 2 time points, during quiescence and during high CMV viremia with colitis. Flow cytometry, using CMV-HLA class I dexamer staining, was performed to quantify CMV-specific CD8 + T cells in comparison to unloaded dexamer control. Furthermore, PBMC were stimulated with CMV antigen in vitro and T cells-IFNg production was analysed by flow cytometry, in comparison to unstimulated and isotype control. RESULTS: At quiescence, 0.2% of total CD8 + T cells was CMV-specific. The percentage increased to 3.64 during high CMV viremia with CMV colitis. At quiescence, upon in vitro CMV stimulation, IFNg-producing CD4 + T cells was 0.35% of total CD4 + T cells and IFNg-producing CD8 + T cells was 0.22% of total CD8 + T cells. However, during high CMV viremia with colitis, only 0.1% of CD4 + T cells and 0.03% of CD8 + T cells produced IFNg in response to in vitro CMV stimulation. CONCLUSIONS: The Good's syndrome patient's CMV-specific T cells number increased more than 15 folds during active CMV infection. However, after in vitro CMV stimulation, T cells from active CMV infection showed lower IFNg production than those in quiescence.

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