title: Late-onset fatal Epstein-Barr virus-associated hemophagocytic syndrome following cord blood cell transplantation for adult acute lymphoblastic leukemia.
abstract: A 43-year-old Japanese woman underwent unrelated cord blood transplantation (CBT) during remission for acute lymphoblastic leukemia with t(4; 11)(q21;q23). Tacrolimus was given for prophylaxis of graft-versus-host disease. The posttransplantation clinical course was mostly uneventful, and the leukemia remained in remission. Fourteen months after CBT, the patient developed pancytopenia and hepatic dysfunction with persistent high-grade fever. The bone marrow was hypocellular with increased numbers of macrophages and hemophagocytes. The numbers of Epstein-Barr virus (EBV) copies in peripheral blood samples were remarkably high. Although the patient showed complete donor-type hematopoiesis, the titer of viral capsid antigen immunoglobulin G was low, and the results of a test for EBV nuclear antigen were negative. There was no clinical response to the reduction of immunosuppressive therapy or to the administration of high-dose methylprednisolone, human immunoglobulin, or acyclovir. The patient died 466 days after CBT of massive gastrointestinal hemorrhage due to bone marrow and hepatic failures. This case demonstrates that fatal EBV-associated hemophagocytic syndrome (HPS) can occur more than 1 year after CBT. This report is the first of a case of late-onset EBV-associated HPS following CBT.
authors.affiliation: Division of Hematology and Oncology, Department of Medicine, Akita University School of Medicine, Akita, Japan.
authors.forename: Yoshinari
authors.initials: Y
authors.lastname: Kawabata
authors.forename: Makoto
authors.initials: M
authors.lastname: Hirokawa
authors.forename: Yoshinobu
authors.initials: Y
authors.lastname: Saitoh
authors.forename: Shigeki
authors.initials: S
authors.lastname: Kosugi
authors.forename: Tomoko
authors.initials: T
authors.lastname: Yoshioka
authors.forename: Masumi
authors.initials: M
authors.lastname: Fujishima
authors.forename: Naohito
authors.initials: N
authors.lastname: Fujishima
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Kameoka
authors.forename: Hirobumi
authors.initials: H
authors.lastname: Saitoh
authors.forename: Masaaki
authors.initials: M
authors.lastname: Kume
authors.forename: Naoto
authors.initials: N
authors.lastname: Takahashi
authors.forename: Ken-ichi
authors.initials: K
authors.lastname: Sawada
mesh_terms: D000212:Acyclovir / Q000008:administration & dosage; D000328:Adult; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D000914:Antibodies, Viral / Q000097:blood; D000998:Antiviral Agents / Q000008:administration & dosage; D001855:Bone Marrow Diseases / Q000097:blood / Q000188:drug therapy / Q000209:etiology / Q000821:virology; D020031:Epstein-Barr Virus Infections / Q000097:blood / Q000188:drug therapy / Q000209:etiology; D019309:Epstein-Barr Virus Nuclear Antigens / Q000097:blood; D005260:Female; D006410:Hematopoiesis; D006470:Hemorrhage / Q000097:blood / Q000188:drug therapy / Q000209:etiology / Q000821:virology; D004854:Herpesvirus 4, Human; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D017093:Liver Failure / Q000097:blood / Q000188:drug therapy / Q000209:etiology / Q000821:virology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000097:blood / Q000188:drug therapy / Q000209:etiology / Q000821:virology; D008775:Methylprednisolone / Q000008:administration & dosage; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000150:complications / Q000628:therapy*; D013997:Time Factors; D018183:Transplantation Chimera
chemical_list: D000893:Anti-Inflammatory Agents; D000914:Antibodies, Viral; D000998:Antiviral Agents; D019309:Epstein-Barr Virus Nuclear Antigens; D007074:Immunoglobulin G; D000212:Acyclovir; D008775:Methylprednisolone