SH2D1A and hemophagocytic syndrome: Susceptibility to EBV-driven HLH may be seen in mutations of SH2D1A, causing X-linked lymphoproliferative syndrome (XLP) 1 and manifesting in hypogammaglobulinemia, and development of EBV-driven lymphoma, as well as other IEIs rendering patients unable to handle this virus effectively, such as deficiencies of CD27, CTPS1, ITK, and CD70 (146, 147).