title: Epstein-Barr virus-associated lymphoproliferative disorder after unrelated bone marrow transplantation in a young child with Wiskott-Aldrich syndrome.
abstract: We report a case of a 16-month-old Wiskott-Aldrich syndrome (WAS) patient with a WASP gene mutation who received human leukocyte antigen (HLA)-matched, unrelated allogeneic bone marrow transplantation (BMT) followed by an Epstein-Barr virus-associated lymphoproliferative disorder (EB-LPD), diagnosed by clinical findings, polymerase chain reaction detection of the EB virus genome, and spontaneous lymphocyte proliferation of donor cell origin. EB-LPD is one of frequent lethal complications in HLA-mismatched or unrelated BMT in this syndrome. Adoptive immunotherapy with donor leukocyte transfusion, including appropriate numbers of CD3-positive T cells, was effective for the EB-LPD, achieving almost complete recovery 1 year later without any findings of graft-versus-host disease.
authors.affiliation: Department of Pediatric Oncology, Tohoku University, Sendai, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Sasahara
authors.forename: S
authors.initials: S
authors.lastname: Kawai
authors.forename: M
authors.initials: M
authors.lastname: Itano
authors.forename: S
authors.initials: S
authors.lastname: Kumaki
authors.forename: H
authors.initials: H
authors.lastname: Fujie
authors.forename: M
authors.initials: M
authors.lastname: Minegishi
authors.forename: S
authors.initials: S
authors.lastname: Tsuchiya
authors.forename: T
authors.initials: T
authors.lastname: Konno
mesh_terms: D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D006566:Herpesviridae Infections / Q000209:etiology*; D004854:Herpesvirus 4, Human; D006801:Humans; D007223:Infant; D017708:Leukocyte Transfusion; D008232:Lymphoproliferative Disorders / Q000209:etiology*; D008297:Male; D014412:Tumor Virus Infections / Q000209:etiology*; D014923:Wiskott-Aldrich Syndrome / Q000150:complications / Q000628:therapy*