title: Outcome for children after failed transplant for primary haemophagocytic lymphohistiocytosis.
abstract: Primary haemophagocytic lymphohistiocytosis is a rare disorder of childhood, which is usually fatal without allogeneic stem cell transplantation (SCT). For children who lack a matched family or closely matched unrelated donor, SCT using haploidentical parental stem cells has been used, but is associated with an increased risk of graft failure. The most appropriate subsequent management for those children who survive after graft rejection is currently unclear. We report the outcome for three such children. After a period of disease quiescence lasting 4 months to 8 years, disease recurrence and subsequent death occurred in each case. Accordingly, a second SCT is recommended.
authors.affiliation: Department of Haematology, Great Ormond Street Hospital for Children NHS Trust, London WC1N 3JH, UK.
authors.forename: K M
authors.initials: KM
authors.lastname: Ardeshna
authors.forename: J
authors.initials: J
authors.lastname: Hollifield
authors.forename: J M
authors.initials: JM
authors.lastname: Chessells
authors.forename: P
authors.initials: P
authors.lastname: Veys
authors.forename: D K
authors.initials: DK
authors.lastname: Webb
mesh_terms: D005260:Female; D005500:Follow-Up Studies; D018380:Hematopoietic Stem Cell Transplantation; D015616:Histiocytosis, Non-Langerhans-Cell / Q000188:drug therapy / Q000601:surgery*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D008297:Male; D014184:Transplantation, Homologous; D017211:Treatment Failure
chemical_list: D007166:Immunosuppressive Agents