title: Epstein-Barr Virus-positive T-cell Lymphoproliferative Disease Following Umbilical Cord Blood Transplantation for Acute Myeloid Leukemia.
abstract: We report a case of the extremely rare condition Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disease (LPD) which occurred after umbilical cord blood transplantation. A 25-year-old Japanese man underwent cord blood transplantation from a male human leukocyte antigen 4/6-matched donor due to acute myeloid leukemia with trisomy 8. Bone marrow examination on day 30 showed chimerism with at least 90% donor cells and complete hematological response. Chronic symptoms of graft-versus-host disease appeared only on the skin and were successfully treated with cyclosporine alone. Three years later, however, the patient experienced repeated cold-like symptoms and was hospitalized with liver dysfunction. A high fever developed and was followed by significant edema of the right side of the face. The EBV DNA copy number in whole peripheral blood was 2×10(4)/mL. Liver biopsy showed invasion of EBV-infected CD8-positive T cells. Southern blotting analysis of the whole peripheral blood showed that the T-cell receptor Cβ1 rearrangement was positive. On the basis of these results, EBV-positive T-cell LPD was diagnosed and treated with prednisolone, cyclosporine, and etoposide, followed by cyclophosphamide, doxorubicin, vincristine, and prednisone. However, the patient died of cardiac function failure, pneumonia, and pulmonary hemorrhage, all of unidentified cause. Most cases of EBV-related LPD after hematopoietic stem cell transplantation consist of EBV-positive B-cell LPD, and, to our knowledge, de novo EBV-positive T-cell LPD subsequent to transplantation has not been previously reported.
authors.affiliation: Department of Hematology, Nippon Medical School.
authors.forename: Shunsuke
authors.initials: S
authors.lastname: Yui
authors.forename: Hiroki
authors.initials: H
authors.lastname: Yamaguchi
authors.forename: Ken-ichi
authors.initials: K
authors.lastname: Imadome
authors.forename: Ayako
authors.initials: A
authors.lastname: Arai
authors.forename: Mikiko
authors.initials: M
authors.lastname: Takahashi
authors.forename: Ryuji
authors.initials: R
authors.lastname: Ohashi
authors.forename: Hayato
authors.initials: H
authors.lastname: Tamai
authors.forename: Keiichi
authors.initials: K
authors.lastname: Moriya
authors.forename: Kazutaka
authors.initials: K
authors.lastname: Nakayama
authors.forename: Akira
authors.initials: A
authors.lastname: Shimizu
authors.forename: Koiti
authors.initials: K
authors.lastname: Inokuchi
mesh_terms: D000328:Adult; D018414:CD8-Positive T-Lymphocytes / Q000473:pathology / Q000821:virology*; D036101:Cord Blood Stem Cell Transplantation / Q000009:adverse effects*; D020031:Epstein-Barr Virus Infections / Q000209:etiology*; D017809:Fatal Outcome; D004854:Herpesvirus 4, Human; D006801:Humans; D015470:Leukemia, Myeloid, Acute / Q000601:surgery*; D008099:Liver / Q000473:pathology / Q000821:virology; D008232:Lymphoproliferative Disorders / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology / Q000821:virology; D008297:Male; D011183:Postoperative Complications; D055815:Young Adult