Authors
Joseph Hai Oved
Published in
Journal of human immunity. Volume 2. Issue 6. Nov 02, 2026. Epub Oct 05, 2026.
Abstract
Primary immune regulatory disorders (PIRDs), autoinflammatory, and hyperinflammatory syndromes can be life-threatening and/or significantly impact quality of life. Targeted immune modulation may control symptoms for some, but allogeneic hematopoietic stem cell transplantation (alloHCT) remains the only cure for many patients. Despite advances in alloHCT, outcomes for this population continue to need optimization. Focus areas include referral timing, patient selection, multidisciplinary evaluation, targeted pretransplant immune suppression (PTIS), graft selection, model- and exposure-based conditioning, and post-alloHCT management. Here, we describe our preferred alloHCT approach for this patient population. This includes early alloHCT referral, continued evaluation to identify an appropriate alloHCT window, 6-12 wk of targeted PTIS for inflamed patients, a myeloablative model-based reduced-toxicity conditioning regimen, a strong index of suspicion for posttransplant immune dysregulation, and multidisciplinary long-term follow-up. Emphasis is placed on the need for predictive biomarkers to guide clinical decision-making, objective measurements of long-term response/disease resolution, and prospective multicenter trials. By refining our approaches, curative outcomes will continue to improve.
PMID:
42831798
Bibliographic data and abstract were imported from PubMed on 05 Oct 2026.
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