CD40LG and lymphopenia: According to an immunological point of view, she showed: hypergammaglobulinaemia (IgG 19.9 g/l) at onset, with normal IgM and IgA, and absolute lymphopenia (1,543/mmc with n.v. 1,900–3,700), with a significant memory T cells deficiency more marked on CD8+CD45RO+ (16/mmc with n.v. 30–330) but involving also CD4+CD45RO+ T cells (105/mmc with n.v. 70–390) with naïve T cells showing a clear prevalence in the peripheral blood (Table 1).