title: Unrelated bone marrow transplantation induced long-term remission in a patient with life-threatening Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis.
abstract: We present a case of life-threatening Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (HLH) with severe hepatitis that was successfully treated by allogeneic stem cell transplantation from an unrelated donor. A 26-year-old woman was admitted to hospital with a high fever and liver dysfunction. Laboratory tests, including bone marrow aspiration, revealed severe HLH that occurred after EBV infection. High-dose methylprednisolone and etoposide therapy did not control the disease. We could control the HLH, but the EBV viremia continued following the CHOPE (cyclophosphamide, doxorubicin, vincristine, prednisone, and etoposide) chemotherapy regimen. Therefore, the patient underwent allogeneic bone marrow transplantation from an HLA-matched, unrelated donor. The patient has remained in good condition without disease recurrence for 2 years after bone marrow transplantation. Although there is no consensus regarding allogeneic stem cell transplantation for EBV-HLH, it is the treatment of choice for aggressive EBV-HLH when the patient is refractory to intensive chemotherapy.
authors.affiliation: Department of Hematology, Meitetsu Hospital, Nagoya, Japan. akunitom@mx5.suisui-w.ne.jp
authors.forename: Akane
authors.initials: A
authors.lastname: Kunitomi
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kimura
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Ito
authors.forename: Kazuyuki
authors.initials: K
authors.lastname: Naitoh
authors.forename: Nobuhiko
authors.initials: N
authors.lastname: Noda
authors.forename: Hiroatsu
authors.initials: H
authors.lastname: Iida
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Sao
mesh_terms: D000328:Adult; D016026:Bone Marrow Transplantation; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000601:surgery*; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D017403:In Situ Hybridization; D051359:Lymphohistiocytosis, Hemophagocytic / Q000601:surgery* / Q000821:virology; D016133:Polymerase Chain Reaction; D014766:Viremia / Q000601:surgery