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

Abstract

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 × 10⁹/L 0.6–1.2), and 0.5 × 10⁹/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.