title: Outcome of bone marrow transplantation in acquired and inherited aplastic anaemia in the Republic of Ireland.
abstract: BACKGROUND
  Severe Aplastic Anaemia (SAA) and Fanconi Anaemia (FA) are rare haematological disorders characterised by pancytopenia and bone marrow hypoplasia.
  
  
  OBJECTIVE
  We performed a retrospective study of all patients who underwent BMT for SAA and FA at St James's Hospital, Dublin, and at OLHSC, Crumlin, between 1985 and 2002.
  
  
  METHODS
  The medical records of 63 patients, 50 with acquired SAA and 13 with FA, were reviewed.
  
  
  RESULTS
  The median age at the time of transplant was 14 years (range 3-43 years). The actuarial survival (OS) (n = 63) was 76% at 17 years. The transplant related mortality (TRM) was 22% (n = 14). The most common cause of death was infection (46%). The survival was significantly better in patients receiving their transplant after 1995 (p = 0.002). Outcome was superior in those receiving less than 20 red cell transfusions prior to transplant: OS 91% (< 20 Units) versus 62% (> or = 20 Units).
  
  
  CONCLUSIONS
  These national results are comparable to those of published international series and support the use of BMT in the treatment of SAA and FA. The known adverse effect of prior transfusion was confirmed.
authors.affiliation: Trinity Centre, St James's Hospital, Dublin. andrea.piccin@ibts.ie
authors.forename: A
authors.initials: A
authors.lastname: Piccin
authors.forename: A
authors.initials: A
authors.lastname: O'Marcaigh
authors.forename: O
authors.initials: O
authors.lastname: Smith
authors.forename: J
authors.initials: J
authors.lastname: O'Riordan
authors.forename: M
authors.initials: M
authors.lastname: Crowley
authors.forename: E
authors.initials: E
authors.lastname: Vandenberg
authors.forename: N
authors.initials: N
authors.lastname: Gardiner
authors.forename: S
authors.initials: S
authors.lastname: McCann
mesh_terms: D000293:Adolescent; D000328:Adult; D000741:Anemia, Aplastic / Q000235:genetics / Q000401:mortality / Q000601:surgery*; D016026:Bone Marrow Transplantation; D002648:Child; D002675:Child, Preschool; D005199:Fanconi Anemia / Q000401:mortality / Q000628:therapy; D005260:Female; D006801:Humans; D007494:Ireland; D008297:Male; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome