title: Cord Blood Transplantation Following Reduced-intensity Conditioning for Adult-onset Inherited Hemophagocytic Lymphohistiocytosis.
abstract: Inherited hemophagocytic lymphohistiocytosis (HLH) is a genetic anomaly disorder in which abnormally activated cytotoxic T lymphocytes cannot induce the apoptosis of target cells and antigen-presenting cells, leading to hemophagocytosis, pancytopenia, and a variety of symptoms such as a high fever. The present patient with adult-onset HLH developed refractory disease despite receiving immunosuppressive treatments. He underwent a reduced-intensity conditioning (RIC) regimen that comprised antithymocyte globulin (ATG) followed by cord blood transplantation (RIC-CBT). He achieved and maintained a complete donor type. The incorporation of ATG into RIC-CBT may prevent graft failure and control hemophagocytosis, however, further efforts are necessary to reduce infectious complications.
authors.affiliation: Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, Japan.
authors.forename: Takuro
authors.initials: T
authors.lastname: Kuriyama
authors.forename: Koji
authors.initials: K
authors.lastname: Kato
authors.forename: Keiji
authors.initials: K
authors.lastname: Sakamoto
authors.forename: Masayasu
authors.initials: M
authors.lastname: Hayashi
authors.forename: Shuichiro
authors.initials: S
authors.lastname: Takashima
authors.forename: Yasuo
authors.initials: Y
authors.lastname: Mori
authors.forename: Katsuto
authors.initials: K
authors.lastname: Takenaka
authors.forename: Hiromi
authors.initials: H
authors.lastname: Iwasaki
authors.forename: Takanori
authors.initials: T
authors.lastname: Teshima
authors.forename: Naoki
authors.initials: N
authors.lastname: Harada
authors.forename: Koji
authors.initials: K
authors.lastname: Nagafuji
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Miyamoto
authors.forename: Koichi
authors.initials: K
authors.lastname: Akashi
mesh_terms: D017668:Age of Onset; D000368:Aged; D000961:Antilymphocyte Serum / Q000627:therapeutic use*; D036101:Cord Blood Stem Cell Transplantation; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000628:therapy* / Q000821:virology; D017809:Fatal Outcome; D006086:Graft vs Host Disease / Q000188:drug therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis / Q000235:genetics / Q000601:surgery*; D008297:Male; D013602:T-Lymphocytes, Cytotoxic; D018183:Transplantation Chimera; D019172:Transplantation Conditioning / Q000379:methods
chemical_list: D000961:Antilymphocyte Serum; D007166:Immunosuppressive Agents