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

IF 4.5 3区医学 Q2 IMMUNOLOGY

Clinical immunology Pub Date : 2024-05-23 DOI:10.1016/j.clim.2024.110263

Salem Al-Tamemi , Abdulhakim Al-Rawas , Murtadha Al-Khabori , Khalil Al-Farsi , Mohammed Al-Huneini , Amr Abdalla , Salam Al-Kindi , David Dennison

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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.

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阿曼先天性免疫错误患者造血干细胞移植后的免疫重建和存活率

背景造血干细胞移植（HSCT）是治疗某些先天性免疫错误的一种治愈性疗法。方法对40名接受造血干细胞移植的免疫缺陷患者进行了一项为期17年的回顾性队列研究。供体主要是匹配的同胞供体（60%）。90%和85%的患者分别接受了调理和移植物抗宿主病（GVHD）预防。最后一次随访时的平均供体嵌合率为 88.6% ± 17.9% (40-100)。血清免疫球蛋白（Ig）G水平、CD4+ T细胞计数和CD19+ B细胞计数的中位数分别为11.7克/升（9.2-13.6）、0.9×109/升（0.6-1.2）和0.5×109/升（0.2-0.7）。29名患者（72.5%）接受了静脉注射免疫球蛋白（IVIG）治疗，中位持续时间为10.0个月（4.0-14.0）。移植后随访的中位数为 6.5 年（IQR:1.4-11.5）。结论IRC的监测对于确保足够的无病生存期非常重要。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Clinical immunology 医学-免疫学

CiteScore

12.30

自引率

1.20%

发文量

212

审稿时长

34 days

期刊介绍： Clinical Immunology publishes original research delving into the molecular and cellular foundations of immunological diseases. Additionally, the journal includes reviews covering timely subjects in basic immunology, along with case reports and letters to the editor.