title: Treatment of donor T cell-mediated hematopoietic suppression after haploidentical bone marrow transplantation by T cell modulation in a patient with severe combined immunodeficiency.
abstract: An 8-month-old male patient with severe combined immunodeficiency syndrome was transplanted with maternal, haploidentical T cell-depleted bone marrow without prior conditioning therapy. Acute graft-versus-host disease developed 2 weeks post bone marrow transplantation (BMT) and was successfully treated with cortisone. After cortisone withdrawal the patient developed myeloid and B cell depression concomitant with T cell activation. For specific T cell modulation, treatment with the T cell receptor (TCR) alpha beta chain-binding MoAb BMA031 was initiated in combination with cyclosporin A. GM-CSF was given to enhance myeloid reconstitution. About 1 year post BMT, B cell and granulocyte counts were within the normal range with stable chimerism in both lineages. B cell proliferation tests were normal and first signs of in vitro immunoglobulin synthesis occurred.
authors.affiliation: Department of Pediatric, Hematology and Oncology, Heinrich Heine University Medical Center, Düsseldorf, Germany.
authors.forename: D
authors.initials: D
authors.lastname: Dilloo
authors.forename: H
authors.initials: H
authors.lastname: Hanenberg
authors.forename: E M
authors.initials: EM
authors.lastname: Schneider
authors.forename: M
authors.initials: M
authors.lastname: Hauch
authors.forename: W
authors.initials: W
authors.lastname: Nürnberger
authors.forename: B M
authors.initials: BM
authors.lastname: Zimmer
authors.forename: R
authors.initials: R
authors.lastname: Vöhringer
authors.forename: P
authors.initials: P
authors.lastname: Wernet
authors.forename: U
authors.initials: U
authors.lastname: Göbel
authors.forename: S E
authors.initials: SE
authors.lastname: Burdach
mesh_terms: D000380:Agranulocytosis / Q000209:etiology / Q000628:therapy; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D001402:B-Lymphocytes / Q000276:immunology; D016026:Bone Marrow Transplantation / Q000009:adverse effects* / Q000276:immunology; D006086:Graft vs Host Disease / Q000209:etiology / Q000628:therapy; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D008297:Male; D016693:Receptors, Antigen, T-Cell, alpha-beta; D016511:Severe Combined Immunodeficiency / Q000276:immunology / Q000601:surgery* / Q000628:therapy; D013601:T-Lymphocytes / Q000276:immunology*
chemical_list: D000911:Antibodies, Monoclonal; D016693:Receptors, Antigen, T-Cell, alpha-beta; D016178:Granulocyte-Macrophage Colony-Stimulating Factor