title: Epstein-Barr virus associated post-transplantation lymphoproliferative disorder with hemophagocytosis in a child with Wiskott-Aldrich syndrome.
abstract: A 23-month-old boy with Wiskott-Aldrich syndrome (WAS) received human leukocyte antigen (HLA)-one locus mismatched, unmanipulated allogeneic bone marrow graft from his mother. An Epstein-Barr virus (EBV)-associated post-transplantation lymphoproliferative disorder (PTLD) of donor cell origin and hemophagocytosis syndrome with fever, lymphadenopathy, hepatosplenomegaly, seizures, involuntary movements and pancytopenia developed 52 days after transplantation. It was difficult to decide on the treatment strategy because the patient presented with B-cell hyperplasia that morphologically appeared malignant but was oligoclonal by heavy-chain analyses. Despite of donor leukocyte transfusion, low dose chemotherapy, and anti-B cell monoclonal antibody immunotherapy, the patient died 107 days after transplantation.
authors.affiliation: Department of Pediatrics, Taipei Hospital, Taipei, Taiwan, Republic of China.
authors.forename: I Jen
authors.initials: IJ
authors.lastname: Wang
authors.forename: Meng Yao
authors.initials: MY
authors.lastname: Lu
authors.forename: Bor Leun
authors.initials: BL
authors.lastname: Chiang
authors.forename: Wei Chou
authors.initials: WC
authors.lastname: Lin
authors.forename: Don Tsamn
authors.initials: DT
authors.lastname: Lin
authors.forename: Kai-Hsin
authors.initials: KH
authors.lastname: Lin
mesh_terms: D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D020031:Epstein-Barr Virus Infections / Q000209:etiology* / Q000473:pathology; D017809:Fatal Outcome; D015616:Histiocytosis, Non-Langerhans-Cell / Q000209:etiology* / Q000473:pathology; D006801:Humans; D007223:Infant; D008232:Lymphoproliferative Disorders / Q000209:etiology* / Q000473:pathology; D008297:Male; D014057:Tomography, X-Ray Computed; D014923:Wiskott-Aldrich Syndrome