title: Haploidentical peripheral blood stem cell transplantation with a combination of CD34 selection and T cell depletion as graft-versus-host disease prophylaxis in a patient with severe combined immunodeficiency.
abstract: Severe combined immunodeficiencies are a group of rare genetic disorders characterized by a profound impairment in both cellular and humoral immune functions. This disorder is rapidly fatal without bone marrow transplantation. Unfortunately, most children lack a histocompatible donor. The development of T cell depletion allows for haploidentical transplantation with reduced risk of GVHD. In the present article, we discuss the case of a child diagnosed with X-linked severe combined immunodeficiency for whom haploidentical peripheral blood stem cell transplantation was performed by selecting CD34-positive cells followed by depletion of T cells. Both selection and purging were performed using immunoselection by the biotin-avidin system (CEPRATE System). The CD34-enriched T cell-depleted product contained 5.05 x 10(6)/kg CD34+ cells with only 0.01 [corrected] x 10(6)/kg CD3+ cells, achieving a T cell depletion of 4.2 log.
authors.affiliation: Department of Hematology, Hospital La Paz, Madrid, Spain.
authors.forename: M P
authors.initials: MP
authors.lastname: Martín-Hernández
authors.forename: R
authors.initials: R
authors.lastname: Arrieta
authors.forename: A
authors.initials: A
authors.lastname: Martínez
authors.forename: P
authors.initials: P
authors.lastname: García
authors.forename: V
authors.initials: V
authors.lastname: Jimenez-Yuste
authors.forename: F
authors.initials: F
authors.lastname: Hernandez-Navarro
mesh_terms: D018952:Antigens, CD34; D006086:Graft vs Host Disease / Q000517:prevention & control*; D006239:Haplotypes; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007223:Infant; D008212:Lymphocyte Depletion; D008297:Male; D016511:Severe Combined Immunodeficiency / Q000628:therapy*; D013601:T-Lymphocytes; D019172:Transplantation Conditioning
chemical_list: D018952:Antigens, CD34