title: Donor T-cell-mediated pancytopenia after haploidentical hematopoietic stem cell transplant for severe combined immunodeficiency.
abstract: Haploidentical hematopoietic stem cell transplant with T-cell depletion may result in donor T-cell engraftment in infants with severe combined immunodeficiency disease. Engraftment of other hematopoietic lines is achieved rarely, and pancytopenia and hemophagocytosis as a result of donor T-cell engraftment have not been reported. We report an infant with severe combined immunodeficiency who developed graft versus host disease with pancytopenia as a result of engraftment of maternal T cells after T-depleted hematopoietic stem cell transplant. His pancytopenia resolved after thymoglobulin and a stem cell boost. Thrombocytopenia resolved with rituximab.
authors.affiliation: Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
authors.forename: Robin
authors.initials: R
authors.lastname: Norris
authors.forename: Michele
authors.initials: M
authors.lastname: Paessler
authors.forename: Nancy
authors.initials: N
authors.lastname: Bunin
mesh_terms: D006085:Graft Survival; D006086:Graft vs Host Disease; D006239:Haplotypes; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects / Q000379:methods*; D006801:Humans; D007223:Infant; D008297:Male; D010198:Pancytopenia / Q000628:therapy*; D016511:Severe Combined Immunodeficiency / Q000628:therapy*; D013601:T-Lymphocytes / Q000502:physiology* / Q000637:transplantation; D016896:Treatment Outcome