ArticleJournal of clinical immunology2013
Post-transplantation B cell function in different molecular types of SCID.
Article in Journal of clinical immunology, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 34 papers, 1 of them a synthesis that pooled it.
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Who cites it
34 citing papers in PubMed, 1 synthesis or guideline pooled it.
- B-Cell Immunophenotyping to Predict Vaccination Outcome in the Immunocompromised - A Systematic Review.Frontiers in immunology · 2021Pooled it
- B-cell differentiation and IL-21 response inBlood · 2018Trial
- Trial
- Human lymphoid development in the absence of common γ-chain receptor signaling.Journal of immunology (Baltimore, Md. : 1950) · 2014Trial
- Outcomes following matched sibling donor transplantation for severe combined immunodeficiency: a report from the PIDTC.Blood advances · 2026Article
- Updates in the Understanding of Immunoglobulin Replacement Therapy in Primary Immune Deficiency Disorders: Function, Composition, and Role in Reconstitution and Immunomodulation.Immunology and allergy clinics of North America · 2025Review
- Article
- Normal IgH Repertoire Diversity in an Infant with ADA Deficiency After Gene Therapy.Journal of clinical immunology · 2021Article
- Intact B-Cell Signaling and Function With Host B-Cells 47 Years After Transplantation for X-SCID.Frontiers in immunology · 2020Article
- Immune Reconstitution After Gene Therapy Approaches in Patients With X-Linked Severe Combined Immunodeficiency Disease.Frontiers in immunology · 2020Review
- Novel Engraftment and T Cell Differentiation of Human Hematopoietic Cells inFrontiers in immunology · 2020Article
- Hematopoietic Stem Cell Transplantation for Severe Combined Immunodeficiency (SCID).Frontiers in pediatrics · 2019Review
- SCID genotype and 6-month posttransplant CD4 count predict survival and immune recovery.Blood · 2018Article
- Efficacy of lentivirus-mediated gene therapy in an Omenn syndrome recombination-activating gene 2 mouse model is not hindered by inflammation and immune dysregulation.The Journal of allergy and clinical immunology · 2018Article
- Long-Term Health Outcome and Quality of Life Post-HSCT for IL7Rα-, Artemis-, RAG1- and RAG2-Deficient Severe Combined Immunodeficiency: a Single Center Report.Journal of clinical immunology · 2018Article
- Use of Genetic Testing for Primary Immunodeficiency Patients.Journal of clinical immunology · 2018Review
- Long term outcomes of severe combined immunodeficiency: therapy implications.Expert review of clinical immunology · 2017Review
- Recommendations for Screening and Management of Late Effects in Patients with Severe Combined Immunodeficiency after Allogenic Hematopoietic Cell Transplantation: A Consensus Statement from the Second Pediatric Blood and Marrow Transplant Consortium International Conference on Late Effects after Pediatric HCT.Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation · 2017Review
- Unconditioned unrelated donor bone marrow transplantation for IL7Rα- and Artemis-deficient SCID.Bone marrow transplantation · 2017Article
- Current Knowledge and Priorities for Future Research in Late Effects after Hematopoietic Stem Cell Transplantation (HCT) for Severe Combined Immunodeficiency Patients: A Consensus Statement from the Second Pediatric Blood and Marrow Transplant Consortium International Conference on Late Effects after Pediatric HCT.Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation · 2017Review
Corrections and comments
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Authors and funding
6 authors.
Funding
Abstract
purposeSevere combined immunodeficiency (SCID) is a syndrome of diverse genetic cause characterized by profound deficiencies of T, B and sometimes NK cell function. Non-ablative HLA-identical or rigorously T cell-depleted haploidentical parental bone marrow transplantation (BMT) results in thymus-dependent genetically donor T cell development in the recipients, leading to a high rate of long-term survival. However, the development of B cell function has been more problematic. We report here results of analyses of B cell function in 125 SCID recipients prior to and long-term after non-ablative BMT, according to their molecular type.
methodsStudies included blood immunoglobulin measurements; antibody titers to standard vaccines, blood group antigens and bacteriophage Φ X 174; flow cytometry to examine for markers of immaturity, memory, switched memory B cells and BAFF receptor expression; B cell chimerism; B cell spectratyping; and B cell proliferation.
resultsThe results showed that B cell chimerism was not required for normal B cell function in IL7Rα-Def, ADA-Def and CD3-Def SCIDs. In X-linked-SCID, Jak3-Def SCID and those with V-D-J recombination defects, donor B cell chimerism was necessary for B cell function to develop.
conclusionThe most important factor determining whether B cell function develops in SCID T cell chimeras is the underlying molecular defect. In some types, host B cells function normally. In those molecular types where host B cell function did not develop, donor B cell chimerism was necessary to achieve B cell function. 236 words.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.