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Morbidity is high, and disease manifestations can be life-threatening. Curative allogeneic hematopoietic stem cell transplantation (HSCT) historically has had poor outcomes. We identified 36 patients with STAT1 GOF disease, receiving 40 HSCT procedures in 2010\u20132023, in a combined effort of the EBMT-IEWP and the PIDTC. Median age at first transplant was 11 years (range 1\u201333). Indications for HSCT were combined immunodeficiency, severe and\/or refractory infections, and autoimmunity. Acute GvHD occurred in 22\/40 HSCT procedures; 5 patients suffered from grade III\/IV acute GvHD. One patient had chronic GvHD. Overall survival was 72.2%, and event-free survival was 55.6%, markedly improved from an earlier report on HSCT for STAT1 GOF disease. Patients with an HCT-CI score of 1 or higher had worse outcome. 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