title: Retrospective survey on the prevalence and outcome of prior autoimmune diseases in patients with aplastic anemia reported to the registry of the European group for blood and marrow transplantation.
abstract: BACKGROUND
  Aplastic anemia (AA) is rarely described after a diagnosis of autoimmune disease (aID).
  
  
  OBJECTIVE
  To assess the prevalence of prior aID in patients with AA recorded in the registry of the European Group for Blood and Marrow Transplantation (EBMT) and to evaluate treatment and outcome.
  
  
  METHODS
  1,251 AA patients from 18 EBMT centers were assessed.
  
  
  RESULTS
  Fifty patients (4%) were eligible: 22 males and 28 females with a median age of 46 years at the diagnosis of aID and of 51 years at the diagnosis of AA. Information on the treatment of AA was available in 49 patients: 38 received only immunosuppressive therapy (IST), 8 patients underwent hematopoietic stem cell transplantation (HSCT) - 6 as first-line therapy and 2 after failure of IST - whilst 3 patients had a spontaneous recovery. After a median follow-up of 3.19 years, 32 patients were alive, including 7 of the 8 patients who underwent HSCT. Only 6 of 32 patients who were alive at the last follow-up were receiving IST for AA.
  
  
  CONCLUSIONS
  Most cases of AA following aID benefitted from IST or HSCT if a matched donor was available. Further prospective investigation is needed to assess the effects of IST on the outcome of underlying aID.
authors.affiliation: Pediatric Hematology Oncology, Department of Pediatrics, University of Padua, Padua, Italy. simone.cesaro@azosp.vr.it
authors.forename: Simone
authors.initials: S
authors.lastname: Cesaro
authors.forename: Judith
authors.initials: J
authors.lastname: Marsh
authors.forename: Gloria
authors.initials: G
authors.lastname: Tridello
authors.forename: Alicia
authors.initials: A
authors.lastname: Rovò
authors.forename: Sebastien
authors.initials: S
authors.lastname: Maury
authors.forename: Barbara
authors.initials: B
authors.lastname: Montante
authors.forename: Tamás
authors.initials: T
authors.lastname: Masszi
authors.forename: Maria Teresa
authors.initials: MT
authors.lastname: Van Lint
authors.forename: Boris
authors.initials: B
authors.lastname: Afanasyev
authors.forename: Arturo
authors.initials: A
authors.lastname: Iriondo Atienza
authors.forename: Marc
authors.initials: M
authors.lastname: Bierings
authors.forename: Cecilia
authors.initials: C
authors.lastname: Carbone
authors.forename: Michael
authors.initials: M
authors.lastname: Doubek
authors.forename: Edoardo
authors.initials: E
authors.lastname: Lanino
authors.forename: Mahmoud
authors.initials: M
authors.lastname: Sarhan
authors.forename: Antonio
authors.initials: A
authors.lastname: Risitano
authors.forename: Katerina
authors.initials: K
authors.lastname: Steinerova
authors.forename: Anders
authors.initials: A
authors.lastname: Wahlin
authors.forename: Anna
authors.initials: A
authors.lastname: Pegoraro
authors.forename: Jakob
authors.initials: J
authors.lastname: Passweg
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000741:Anemia, Aplastic / Q000453:epidemiology* / Q000209:etiology / Q000628:therapy; D001327:Autoimmune Diseases / Q000150:complications / Q000453:epidemiology* / Q000628:therapy; D016026:Bone Marrow Transplantation; D002648:Child; D003625:Data Collection; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012042:Registries; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents