title: Cord Blood Transplantation Following Reduced-Intensity Conditioning for Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis during Systemic Lupus Erythematosus Treatment.
abstract: Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is a serious disorder in which monoclonal growth of T cells infected with EBV and macrophage activation cause pancytopenia, high fever and acute liver failure. Patients with chemotherapy- or immunosuppression-resistant EBV-HLH require allogeneic hematopoietic stem cell transplantation (allo-HSCT), but patients who have no sibling donors may not have time to wait for an unrelated donor. In pediatric patients, there are some reports on allogeneic cord blood transplantation (allo-CBT) for the treatment of EBV-HLH; however, in adult patients, reports of allo-CBT for EBV-HLH are quite limited. The present case of a 20-year-old woman with chemotherapy-resistant EBV-HLH and systemic lupus erythematosus (SLE) who underwent allo-CBT following reduced-intensity conditioning (RIC-CBT). She achieved and maintained a complete donor type and the EBV-DNA load, and the titers of anti-double stranded DNA and antinuclear antibodies became negative. It is therefore considered that RIC-CBT is an effective treatment option for adult onset HLH. However, because the effectiveness of allo-HSCT for SLE remains unclear and transplant-related mortality is high, it is recommended that allo-HSCT for SLE is restricted to patients concomitant with oncohematological disease as with our present case.
authors.affiliation: Department of Internal Medicine, Miyazaki Prefectural Miyazaki Hospital.
authors.forename: Takuro
authors.initials: T
authors.lastname: Kuriyama
authors.forename: Noriaki
authors.initials: N
authors.lastname: Kawano
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Yamashita
authors.forename: Ikuo
authors.initials: I
authors.lastname: Kikuchi
mesh_terms: D000328:Adult; D064591:Allografts; D036101:Cord Blood Stem Cell Transplantation; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000628:therapy; D005260:Female; D004854:Herpesvirus 4, Human; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000628:therapy / Q000821:virology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology / Q000628:therapy / Q000821:virology; D019172:Transplantation Conditioning