...the outcome of HSCT in patients with syndromes with predominant autoimmunity is unclear, given that target antigens of autoimmune reactions remain unchanged, and both autoimmunity and inflammation can persist because of disease-causing factors extrinsic to the hematopoietic and immune system. ...the need for immunosuppressive treatment decreased, and most patients were off immunoglobulin replacement after HSCT (Fig 1, D and E). The risk of LRBA-related lymphoma or immunosuppression-associated malignancy would be anticipated to be substantially reduced or abolished in HSCT survivors, as well as the future risk of infections, compared with patients undergoing continuous treatment with CTLA4-Ig, sirolimus, or other immunosuppression. ...based on the present results, we would proceed to HSCT if a suitable donor was present. ...among the small cohort of 12 LRBA-deficient patients who underwent transplantation from heterozygous LRBA mutation carriers (n = 6) or LRBA wild-type (n = 1) or unrelated donors (n = 5), no correlation between residual or recurring LRBA-related symptoms and donor LRBA status was detected, arguing against a dose effect of LRBA.2,6 Lastly, these data indicate that any alluded LRBA-dependent effects outside the (transplanted) hematopoietic/immune tissues play no or only an inferior role with regard to outcome and remission.