DOCK8 and hyper-IgE syndrome: The improved understanding of the immunopathology resulted in treatment optimization1–4, such as the benefit of immunoglobulin substitution therapy in addition to rigorous antibiotic treatments due to the discovered impaired adaptive immunity5,11 and the consensus of early hematopoietic stem cell transplantation (HSCT) as treatment of choice in HIES caused by DOCK8 deficiency (DOCK8-HIES)11–13.