Frequency of sepsis in splenectomized ALPS patients and an analysis of risk factors. (A) Sepsis-free survival in 33 splenectomized ALPS patients. (B) Risk of sepsis as a function of age at splenectomy (<5 years or ≥5 years). In each column, the number of patients with sepsis is presented in gray (5 out of 7 patients splenectomized before the age of 5, and 5 out of 26 patients splenectomized at the age of 5 or older). The risk is 12.1 and 1.1 per 100 patient years of follow-up, respectively. (C) Risk of sepsis as a function of compliance with recommended prophylaxis (antibiotic prophylaxis for at least 5 years in children and at least 2 years in adults and up-to-date vaccination schedules for S pneumoniae, H influenzae, and N meningitidis). In each column, the number of patients with sepsis is presented in gray (4 out of 6 noncompliant patients and 6 out of 23 compliant patients). The risk is 5.8 and 3.4 per 100 patient years of follow-up, respectively.