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Outcome of Non-hematological Autoimmunity After Hematopoietic Cell Transplantation in Children with Primary Immunodeficiency.
- Source :
-
Journal of clinical immunology [J Clin Immunol] 2021 Jan; Vol. 41 (1), pp. 171-184. Date of Electronic Publication: 2020 Nov 03. - Publication Year :
- 2021
-
Abstract
- Purpose: Knowledge of post-hematopoietic cell transplantation (HCT) non-hematological autoimmune disease (AD) is far from satisfactory.<br />Method: This multicenter retrospective study focuses on incidence, risk factors, and outcomes of post-HCT AD in 596 children with primary immunodeficiency (PID) who were transplanted from 2009 to 2018.<br />Results: The indications of HCT were severe combined immunodeficiency (SCID, n = 158, 27%) and non-SCID PID (n = 438, 73%). The median age at HCT was 2.3 years (range, 0.04 to 18.3 years). The 5-year overall survival for the entire cohort was 79% (95% cumulative incidence (CIN), 74-83%). The median follow-up of surviving patients was 4.3 years (0.08 to 14.7 years). The CIN of post-HCT AD was 3% (2-5%) at 1 year post-HCT, 7% (5-11%) at 5 years post-HCT, and 11% (7-17%) at 8 years post-HCT. The median onset of post-HCT AD was 2.2 years (0.12 to 9.6 years). Autoimmune thyroid disorder (n = 19, 62%) was the most common post-HCT AD, followed by neuromuscular disorders (n = 7, 22%) and rheumatological manifestations (n = 5, 16%). All patients but one required treatment for post-HCT AD. After multivariate analysis, age at transplant (p = 0.01) and T cell-depleted graft (p < 0.001) were significant predictors of post-HCT AD. None of the T cell-depleted graft recipients developed post-HCT AD. Patients with a lower CD3+ count at 6 months post-HCT had a significant higher incidence of post-HCT AD compared to disease controls. Graft-versus-host disease, viral infection, and donor chimerism had no association with post-HCT AD.<br />Conclusion: Post-HCT AD occurred in 11% at 8 years post-HCT and its occurrence was associated with older age at HCT and unmanipulated graft.
- Subjects :
- Adolescent
Autoimmune Diseases diagnosis
Child
Child, Preschool
Disease Management
Disease Susceptibility
Female
Graft vs Host Disease diagnosis
Graft vs Host Disease epidemiology
Graft vs Host Disease etiology
Hematopoietic Stem Cell Transplantation adverse effects
Hematopoietic Stem Cell Transplantation methods
Humans
Immune Reconstitution
Incidence
Infant
Lymphocyte Count
Male
Primary Immunodeficiency Diseases therapy
Prognosis
Retrospective Studies
Risk Factors
Transplantation Chimera
Treatment Outcome
Autoimmune Diseases epidemiology
Autoimmune Diseases etiology
Autoimmunity
Primary Immunodeficiency Diseases complications
Primary Immunodeficiency Diseases epidemiology
Subjects
Details
- Language :
- English
- ISSN :
- 1573-2592
- Volume :
- 41
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Journal of clinical immunology
- Publication Type :
- Academic Journal
- Accession number :
- 33141919
- Full Text :
- https://doi.org/10.1007/s10875-020-00895-3