ArticleFrontiers in immunology2025
Long-term outcome of bone marrow transplantation in NIK deficiency: non-redundant role of non-canonical NF-κB signaling in thymic reconstitution and secondary lymphoid organ development.
Article in Frontiers in immunology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Introduction: Biallelic mutations in Method: We report long-term outcomes of two patients with genetically confirmed NIK deficiency who underwent HSCT. Results: Both patients achieved full donor chimerism and early T-cell reconstitution with normalized CD3 Discussion: These results highlight the importance of long-term immunologic monitoring, including assessments for immune dysregulation and anti-cytokine autoantibodies. Future therapies should consider adjunct strategies such as thymic regeneration or targeted immune modulation to address the underlying architectural defects in this disorder.
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