title: Successful second unrelated donor BMT in a child with juvenile chronic myeloid leukaemia: documentation of chimaerism using the polymerase chain reaction.
abstract: A 3-year old child with juvenile chronic myeloid leukaemia received a T cell-depleted BMT from a male unrelated donor. There was early graft failure associated with increasing splenomegaly and hypersplenism. Splenectomy was performed 53 days post-transplant and was followed by autologous marrow recovery with return of leukaemia. A second unrelated donor BMT was performed 9 months later using T cell-replete marrow from a similarly matched female donor. Grade 2 GVHD involving the skin and gut responded to treatment with steroids. Chimaerism was assessed using Y-specific polymerase chain reaction (PCR) and microsatellites. Samples taken at the time of splenectomy showed no donor marrow engraftment but there was significant engraftment in the spleen. Following the second transplant, donor-type haematopoiesis was documented using a panel of microsatellite probes. The patient remains well 6 months after transplant. Splenectomy should be considered prior to transplant in patients with significant splenomegaly and hypersplenism. Partial chimaerism in the spleen, but not bone marrow, post-BMT, has not previously been documented. PCR technology is a useful and highly sensitive way to assess chimaerism post-BMT and is informative in sex-matched cases, whilst the small amount of material required is advantageous in paediatric patients.
authors.affiliation: Royal Hospital for Sick Children, Bristol, UK.
authors.forename: D H
authors.initials: DH
authors.lastname: Pamphilon
authors.forename: J M
authors.initials: JM
authors.lastname: Cornish
authors.forename: S
authors.initials: S
authors.lastname: Goodman
authors.forename: L
authors.initials: L
authors.lastname: Ball
authors.forename: M
authors.initials: M
authors.lastname: Lawler
authors.forename: S
authors.initials: S
authors.lastname: McCann
authors.forename: P
authors.initials: P
authors.lastname: Humphries
authors.forename: A
authors.initials: A
authors.lastname: Oakhill
mesh_terms: D016026:Bone Marrow Transplantation; D002675:Child, Preschool; D002678:Chimera / Q000235:genetics*; D004276:DNA, Satellite / Q000235:genetics; D005260:Female; D006801:Humans; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000235:genetics / Q000601:surgery*; D008297:Male; D016133:Polymerase Chain Reaction; D012086:Reoperation; D014019:Tissue Donors; D014998:Y Chromosome
chemical_list: D004276:DNA, Satellite