Severe or recurrent disease manifestation with EBV-lymphoproliferative disease may represent an indication for such invasive treatment strategy, though owing to the lack of (published) data, it is currently not clear what the outcome of aHSCT in NF-κB1-mutant PID is with regard to long-term morbidity and survival. The gene discussed is NFKB1; the disease is pelvic inflammatory disease.