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

被引：0

作者：

Al-Tamemi, Salem ^{[1
,6
]}

Al-Rawas, Abdulhakim ^{[2
]}

Al-Khabori, Murtadha ^{[3
]}

Al-Farsi, Khalil ^{[3
]}

Al-Huneini, Mohammed ^{[3
]}

Abdalla, Amr ^{[2
,4
]}

Al-Kindi, Salam ^{[3
,5
]}

Dennison, David ^{[3
]}

机构：

[1] Sultan Qaboos Univ Hosp, Dept Child Hlth, Clin Immunol & Allergy Unit, Muscat, Oman

[2] Sultan Qaboos Univ Hosp, Dept Child Hlth, BMT Unit, Muscat, Oman

[3] Sultan Qaboos Univ Hosp, Dept Hematol, BMT Unit, Muscat, Oman

[4] Cairo Univ, NCI, Pediat Oncol Dept, Cairo, Egypt

[5] Coll Med & Hlth Sci, Muscat, Oman

[6] Sultan Qaboos Univ Hosp, Dept Child Hlth, Muscat, Oman

来源：

CLINICAL IMMUNOLOGY | 2024年 / 264卷

关键词：

Hematopoietic stem cell transplantation; Inborn errors of immunity; Primary immunodeficiency; Immune reconstitution; Oman; BONE-MARROW-TRANSPLANTATION; SEVERE COMBINED IMMUNODEFICIENCY; INTRAVENOUS IMMUNOGLOBULIN; EXPERIENCE; ANTIBODY; INFECTIONS; CHILDREN; BMT;

D O I：

10.1016/j.clim.2024.110263

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

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.

引用

页数：6

共 31 条

[11] EUROPEAN EXPERIENCE OF BONE-MARROW TRANSPLANTATION FOR SEVERE COMBINED IMMUNODEFICIENCY [J].