Immune reconstitution and survival, following hematopoietic stem cell transplantation in Omani patients with inborn errors of immunity

Background: Hematopoietic stem cell transplantation (HSCT) is a curative treatment for certain inborn errors of immunity. Methods: A 17 -year retrospective cohort study was conducted on 40 immunodeficient patients who underwent HSCT. Results: The median age at transplant was 11.0 months (4.6 -61.0). Donors were primarily matched sibling donors (60%). 90% and 85% of patients received conditioning and graft -versus -host disease (GVHD) prophylaxis, respectively. The mean donor chimerism at the last follow-up was 88.6% +/- 17.9% (40 -100). Median serum immunoglobulin (Ig) G level, CD4 + T -cell count, and CD19 + B -cell count were 11.7 g/L (9.2 -13.6), 0.9 x 10 9 /L 0.6 -1.2), and 0.5 x 10 9 /L (0.2 -0.7), respectively. 29 patients (72.5%) received intravenous immunoglobulins (IVIG) therapy, with a median duration of 10.0 months (4.0 -14.0). The median post -transplant follow-up was 6.5 years (IQR:1.4 -11.5). The 10 -year overall probability of survival is 84.3%. Conclusion: Monitoring IRC is important in ensuring adequate disease -free survival.