Blood: Hematopoietic cell transplantation for chronic granuloma disease: 712 cases study

!---- chronic granulated disease (CGD) is a primary immunodeficiency disease that can lead to life-threatening infections and inflammatory complicationsallo-HCT can cure patients, but the transplant indication is still controversialChiesa and others conducted a multi-center retrospective study to analyze 712 CGD patients who performed allo-HCT transplants at the EBMT Center from 1993 to 2018the 712 CGD patients, including 635 children (18 years old) and 77 adultsmedian follow-up for 45 monthsthe median age fortransplant is 7 years (range 0.1-48.6 years)3 Years OS and EFS Kaplan-Meier estimates were 85.7% (95% CI 82.8-88.5) and 75.8% (95% CI 72.3-79.3), respectivelyin MVA, the decrease in survival rates in older years was associated with an increasein incidence of chronic GVHDAlthough adult patients are 76% (95% CI, 66-86) and 69% (95% CI, 57-80) in adult patientsthe use of an antigen mismatch donoris associated with OS and EFS reductionthere was no significant difference in OS in a small group of patients who received donor-derived hematopoietic cell transplantation from mismatched antigens, but EFS is now reduced (HR 3.69, p-0.001)the choice of conditioning options does not affect OS or EFSin general, this study shows that the prognosis of CGD patients after treatment with allo-HCT is excellent, regardless of age, transplant failure rate and mortality rate are lowthe prognosis of older patients and those receiving an antigen mismatch transplant was relatively not goodtransplants should be strongly considered as a treatment option for young patients, especially in the case of a perfectly matched donor