title: Allogeneic bone marrow transplantation for active Epstein-Barr virus-related lymphoproliferative disease and hemophagocytic lymphohistiocytosis in an infant with severe combined immunodeficiency syndrome.
abstract: A 5-month-old male presented with fever, hepatosplenomegaly, leukocytosis with atypical lymphoblasts, anemia and thrombocytopenia. Severe combined imunodeficiency syndrome (T-, B+, NK+), B lymphoproliferative disease and hemophagocytic lymphohistiocytosis triggered by Epstein-Barr virus (EBV) were diagnosed. As his clinical situation deteriorated rapidly, BMT was performed with unmanipulated marrow stem cells from his EBV-positive HLA-identical sister after conditioning with dexamethasone (1.75 mg/kg/day), cyclophosphamide (114 mg/kg) and etoposide (10 mg/kg), with no immunosuppression given post transplant. Engraftment occurred on day 6 with explosive proliferation of donor CD8(+) T cells. The patient died 3 days later from acute respiratory distress syndrome. Autopsy revealed full donor engraftment and no signs of hemophagocytic lymphohistiocytosis or B lymphoproliferative disease. Thus, transplanted T cells can expand very rapidly within days after BMT and clear EBV lymphoproliferative disease and hemophagocytic lymphohistiocytosis.
authors.affiliation: Kinderklinik and Kinderpoliklinik, Dr von Haunersches Kinderspital, University of Munich, Lindwurmstrasse 4, D-80337 Munich, Germany.
authors.forename: I
authors.initials: I
authors.lastname: Schmid
authors.forename: K
authors.initials: K
authors.lastname: Reiter
authors.forename: F
authors.initials: F
authors.lastname: Schuster
authors.forename: U
authors.initials: U
authors.lastname: Wintergerst
authors.forename: R
authors.initials: R
authors.lastname: Meilbeck
authors.forename: T
authors.initials: T
authors.lastname: Nicolai
authors.forename: B H
authors.initials: BH
authors.lastname: Behloradsky
authors.forename: D K
authors.initials: DK
authors.lastname: Stachel
mesh_terms: D000208:Acute Disease; D016026:Bone Marrow Transplantation / Q000379:methods*; D020031:Epstein-Barr Virus Infections / Q000601:surgery*; D017809:Fatal Outcome; D004854:Herpesvirus 4, Human / Q000302:isolation & purification*; D015616:Histiocytosis, Non-Langerhans-Cell / Q000601:surgery*; D006801:Humans; D007223:Infant; D008232:Lymphoproliferative Disorders / Q000175:diagnosis / Q000601:surgery* / Q000821:virology; D008297:Male; D016511:Severe Combined Immunodeficiency / Q000601:surgery* / Q000821:virology; D014184:Transplantation, Homologous