Back to Search
Start Over
Haematopoietic cell transplantation for acute leukaemia and advanced myelodysplastic syndrome in Fanconi anaemia.
- Source :
-
British journal of haematology [Br J Haematol] 2014 Feb; Vol. 164 (3), pp. 384-95. Date of Electronic Publication: 2013 Oct 30. - Publication Year :
- 2014
-
Abstract
- Acute leukaemia or advanced myelodysplastic syndrome (MDS ≥ 5% blasts) in Fanconi anaemia (FA) patients is associated with a poor prognosis. We report 21 FA patients with acute leukaemia or advanced MDS who underwent haematopoietic cell transplantation (HCT) at the University of Minnesota between 1988 and 2011. Six patients had biallelic BRCA2 mutations. Eight patients received pre-transplant cytoreduction, with 3 achieving complete remission. HCT donor source included human leucocyte antigen-matched sibling (n = 2) or alternative donors (n = 19). Neutrophil engraftment was 95% for the entire cohort, and the incidence of acute graft-versus-host disease was 19%. 5-year overall survival (OS) was 33%, with a relapse rate of 24%, with similar OS in patients with biallelic BRCA2 mutations. Our study supports the use of HCT in the treatment of FA patients with acute leukaemia or advanced MDS, however, the role of chemotherapy prior to HCT remains unclear for this population. FA patients with biallelic BRCA2 are unique and may benefit from higher dose chemotherapy relative to other complementation groups.<br /> (© 2013 John Wiley & Sons Ltd.)
- Subjects :
- Acute Disease
Adolescent
Adult
Child
Child, Preschool
Cohort Studies
Fanconi Anemia blood
Female
Humans
Infant
Leukemia blood
Male
Middle Aged
Myelodysplastic Syndromes blood
Prognosis
Retrospective Studies
Treatment Outcome
Young Adult
Fanconi Anemia surgery
Hematopoietic Stem Cell Transplantation methods
Leukemia surgery
Myelodysplastic Syndromes surgery
Subjects
Details
- Language :
- English
- ISSN :
- 1365-2141
- Volume :
- 164
- Issue :
- 3
- Database :
- MEDLINE
- Journal :
- British journal of haematology
- Publication Type :
- Academic Journal
- Accession number :
- 24172081
- Full Text :
- https://doi.org/10.1111/bjh.12634