Furthermore, two 6–16-year-old CVID patients (V4 and V8, Figure 4A) presented a severe clinical course and could be distinguished by TEMRA CD8+ expansion in V4, probably related to persistent viral infections, and TCM CD4+ and TEM CD8+ in V8; patient V8 developed overtime a MAS (Macrophage Activated Syndrome), leading eventually to death. Here, CD8A is linked to macrophage activation syndrome.