B-II-3, the younger sister, had experienced intermittent fevers since the neonatal period but without anemia and on review at our center also had splenomegaly, elevated inflammatory markers and elevated IFI27 expression on peripheral blood interferon gene signature testing (111.13 - 395.87, RR 0.09 – 2.24; Table 1, Figure 1B). Here, IFI27 is linked to Splenomegaly.