Pediatric IPEX-Associated Dermatitis Responds To Dupilumab: Evidence from Skin Transcriptomics and Immune Profiling

J Clin Immunol. 2026 Feb 7. doi: 10.1007/s10875-025-01979-8. Online ahead of print. ABSTRACT PURPOSE: Immunodysregulation, Polyendocrinopathy, Enteropathy, and X-linked (IPEX) syndrome is a rare autoimmune disorder caused by mutations in the FOXP3 gene. Patients with IPEX frequently present with severe dermatitis, diabetes, and enteropathy. This study explores the efficacy of Dupilumab (an anti-IL-4Rα monoclonal antibody) … Read more

αβT/CD19-depleted Allogeneic Stem Cell Transplantation in Adults with Inborn Errors of Immunity

J Clin Immunol. 2026 Feb 3. doi: 10.1007/s10875-025-01978-9. Online ahead of print. ABSTRACT PURPOSE: Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is successful in pediatric patients with inborn errors of immunity (IEI), but its use in adults is complicated by pre-existing organ damage and increased risk of treatment-related mortality. Ex vivo graft engineering using αβTCR/CD19 depletion … Read more

Follicular Helper T Cells and B Cell Maturation in Patients with 22q11.2 Deletion Syndrome and Recurrent Infections

J Clin Immunol. 2026 Feb 3. doi: 10.1007/s10875-026-01987-2. Online ahead of print. ABSTRACT PURPOSE: 22q11.2 Deletion Syndrome has been primarily described as a disorder of T cell production secondary to thymic hypoplasia. However, there is great complexity in the clinical picture with infections, autoimmunity, and inflammation occurring. Emerging evidence suggests that qualitative T cell dysfunction … Read more

Correction to: A Multicentric Clinical Study to Evaluate Pharmacokinetics, Efficacy, and Safety of Immune Globulin Subcutaneous 20% Weekly/Biweekly Dosing in Treatment-Experienced Patients and Loading/Weekly Maintenance Dosing in Treatment-Naïve Patients with Primary Immunodeficiency

J Clin Immunol. 2026 Feb 2;46(1):15. doi: 10.1007/s10875-026-01983-6. NO ABSTRACT PMID:41627598 | DOI:10.1007/s10875-026-01983-6

A Case-Based Literature Review of RELA Associated Inflammatory Diseases

J Clin Immunol. 2026 Jan 27. doi: 10.1007/s10875-025-01968-x. Online ahead of print. ABSTRACT Behçet’s disease (BD) is a chronic inflammatory disorder characterized by recurrent oral aphthous ulcers, genital ulcers, skin lesions, and uveitis. Recent genetic studies have identified monogenic diseases with phenotypes resembling BD, including RELA-associated inflammatory disease (RAID), Haploinsufficiency of A20 (HA20), and otulipenia. … Read more

Antibody Deficiency in Xeroderma Pigmentosum

J Clin Immunol. 2026 Jan 10;46(1):4. doi: 10.1007/s10875-025-01967-y. ABSTRACT We describe a 3-year-old patient with xeroderma pigmentosum (XP) and genetically confirmed XPA deficiency who presented with recurrent infections in early childhood. Immunological assessment revealed mild hypogammaglobulinemia with IgG2 and IgG3 subclass deficiencies, as well as impaired humoral immunity demonstrated by a reduced antibody response to … Read more

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