title: Long-lasting production of new T and B cells and T-cell repertoire diversity in patients with primary immunodeficiency who had undergone stem cell transplantation: a single-centre experience.
abstract: Levels of Kappa-deleting recombination excision circles (KRECs), T-cell receptor excision circles (TRECs), and T-cell repertoire diversity were evaluated in 1038 samples of 124 children with primary immunodeficiency, of whom 102 (54 with severe combined immunodeficiency and 48 with other types of immunodeficiency) underwent hematopoietic stem cell transplantation. Twenty-two not transplanted patients with primary immunodeficiency were used as controls. Only data of patients from whom at least five samples were sent to the clinical laboratory for routine monitoring of lymphocyte reconstitutions were included in the analysis. The mean time of the follow-up was 8 years. The long-lasting posttransplantation kinetics of KREC and TREC production occurred similarly in patients with severe combined immunodeficiency and with other types of immunodeficiency and, in both groups, the T-cell reconstitution was more efficient than in nontransplanted children. Although thymic output decreased in older transplanted patients, the degree of T-cell repertoire diversity, after an initial increase, remained stable during the observation period. However, the presence of graft-versus-host disease and ablative conditioning seemed to play a role in the time-related shaping of T-cell repertoire. Overall, our data suggest that long-term B- and T-cell reconstitution was equally achieved in children with severe combined immunodeficiency and with other types of primary immunodeficiency.
authors.affiliation: CREA, Diagnostics Department, Spedali Civili of Brescia, 25123 Brescia, Italy.
authors.forename: Monica
authors.initials: M
authors.lastname: Valotti
authors.affiliation: CREA, Diagnostics Department, Spedali Civili of Brescia, 25123 Brescia, Italy.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Sottini
authors.affiliation: Department of Pediatric Oncohaematology and Bone Marrow Transplantation, Spedali Civili of Brescia, 25123 Brescia, Italy.
authors.forename: Arnalda
authors.initials: A
authors.lastname: Lanfranchi
authors.affiliation: Department of Pediatric Oncohaematology and Bone Marrow Transplantation, Spedali Civili of Brescia, 25123 Brescia, Italy.
authors.forename: Federica
authors.initials: F
authors.lastname: Bolda
authors.affiliation: CREA, Diagnostics Department, Spedali Civili of Brescia, 25123 Brescia, Italy.
authors.forename: Federico
authors.identifier: 0000-0001-6740-4307
authors.initials: F
authors.lastname: Serana
authors.affiliation: CREA, Diagnostics Department, Spedali Civili of Brescia, 25123 Brescia, Italy.
authors.forename: Diego
authors.initials: D
authors.lastname: Bertoli
authors.affiliation: CREA, Diagnostics Department, Spedali Civili of Brescia, 25123 Brescia, Italy.
authors.forename: Viviana
authors.initials: V
authors.lastname: Giustini
authors.affiliation: CREA, Diagnostics Department, Spedali Civili of Brescia, 25123 Brescia, Italy.
authors.forename: Marion Vaglio
authors.identifier: 0000-0001-5828-4411
authors.initials: MV
authors.lastname: Tessitore
authors.affiliation: CREA, Diagnostics Department, Spedali Civili of Brescia, 25123 Brescia, Italy.
authors.forename: Luigi
authors.initials: L
authors.lastname: Caimi
authors.affiliation: CREA, Diagnostics Department, Spedali Civili of Brescia, 25123 Brescia, Italy.
authors.forename: Luisa
authors.identifier: 0000-0002-7443-769X
authors.initials: L
authors.lastname: Imberti
mesh_terms: D000293:Adolescent; D000328:Adult; D001402:B-Lymphocytes / Q000276:immunology*; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D014644:Genetic Variation; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000276:immunology* / Q000628:therapy; D016130:Immunophenotyping; D038041:Lymphopoiesis / Q000235:genetics; D008297:Male; D011948:Receptors, Antigen, T-Cell / Q000235:genetics*; D011995:Recombination, Genetic; D012189:Retrospective Studies; D013601:T-Lymphocytes / Q000276:immunology*; D055815:Young Adult
chemical_list: D011948:Receptors, Antigen, T-Cell