Fig. 5.

Autoimmunity and hyperinflammation in WHIM syndrome. A Prevalence of individual autoimmune (AI) and hyperinflammatory (HI) complications (frequency as % patients with subtype among all patients with AI or HI complications, n = 14). B Occurrence of autoimmune and hyperinflammatory complications in isolation or combination (frequency reported as above). C Prevalence of single- and multi-lineage cytopenias (frequency reported as above). D Platelet and hemoglobin nadir during flares (symbols representing individual values; median ± SEM shown). E Prevalence of individual treatment strategies used for ITP and AIHA. F Ratio of patients with a treatment response for first line (steroids ± IVIG) and second line (biologicals, immunosuppressives or splenectomy). Treatment response was scored using the following criteria: “non” = no clinical response or side effects were limiting, “partial” = clinical improvement but therapeutic escalation was required, or “full” = clinical improvement with no escalation