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Bone marrow transplant options and preferences in a sickle cell anemia cohort on chronic transfusions.

Abstract

BACKGROUND
Bone marrow transplantation (BMT) using human leukocyte antigen (HLA)-matched sibling donors can be curative for children with sickle cell anemia (SCA). However, minimal data exist regarding availability of HLA-identical matched siblings for transplant-eligible children, and family interest in pursuing transplantation.
METHODS
We retrospectively analyzed a pediatric SCA cohort receiving chronic transfusions between July 2004 and January 2011. Data were analyzed regarding the number of full siblings and half-siblings, availability, and family interest in HLA testing the full siblings, and interest in proceeding with HLAmatched transplantation.
RESULTS
Among 113 patients, 46 (41%) had at least 1 full sibling and 40 (35%) had an unaffected full sibling who could serve as a BMT donor. The families of 23 of these patients (58%) agreed to HLA-type sibling, 8 of whom (35%) were matched. Transfusion indications for families agreeing to HLA typing included stroke (46%) abnormal TCD (29%), acute chest syndrome (21%), and other CNS reasons (4%). Common reasons to decline HLA typing or transplantation included fear of the process, toxicities of the procedure, and comfort with current quality of life on transfusions. Only 8 of 113 (7%) were eligible for matched BMT, and only 3 (3%) underwent HLA-matched transplantation. Two unmatched children received haploidentical transplantation.
CONCLUSIONS
Most families of children with SCA on chronic transfusions choose to proceed with HLA typing. However, when a matched sibling was identified, most families declined to proceed with matched-sibling transplantation. Discussing BMT as a treatment option, offering HLA typing and identifying barriers may improve acceptance of this treatment modality.

Links

  • Aggregator Full Text
  • Authors

    Hansbury EN, Schultz WH, Ware RE, Aygun B

    Source

    Pediatric blood & cancer 58:4 2012 Apr pg 611-5

    MeSH

    Adolescent
    Anemia, Sickle Cell
    Blood Transfusion
    Bone Marrow Transplantation
    Child
    Child, Preschool
    Female
    Histocompatibility Testing
    Humans
    Infant
    Male
    Retrospective Studies
    Transplantation, Homologous

    Pub Type(s)

    Clinical Trial
    Journal Article

    Language

    eng

    PubMed ID

    22435112