Clinical features of inborn errors of immunity predisposing to EBV-induced disease. (A) Proportions of patients with mutations in the indicated genes developing lymphoproliferative disease (LPD)/malignancy, severe IM, and/or HLH. (B) Breakdown of lymphoproliferative disease and the types of malignancies observed in these patients. DLBCL, diffuse large B cell lymphoma; SMT, smooth muscle tumor; T-LPD, T cell lymphoproliferative disease. Data for XLP1 is not included in this graph. (C) Age of onset of initial presenting clinical features for the indicated genotypes. Data for 25 individual patients with XLP1 (SH2D1A mutations) are derived from Pachlopnik Schmid et al.²⁹  The average for these 25 patients (4.9 years) is consistent with the median reported by Booth et al²⁷  for large cohorts of EBV⁺ (4 years; n = 51) and EBV⁻ (3 years; n = 28) XLP1 patients. (D) Survival post-HSCT. Data for XLP1 patients is based on the study by Booth et al²⁷ ; data for the other genotypes are from individual case reports. Note that apart from XLP1 due to SH2D1A mutations, relatively few patients with other monogenic mutations have undergone HSCT.