G. Lanasa et al., UNRELATED BONE-MARROW TRANSPLANTATION IN A WISKOTT-ALDRICH SYNDROME PATIENT SHARING 2 HLA-EXTENDED HAPLOTYPES WITH THE DONOR, Haematologica, 81(2), 1996, pp. 178-181
We report a case of BMT from an unrelated donor (MUD) in a patient aff
ected by Wiskott-Aldrich syndrome (WAS). The donor-recipient pair was
completely identical for two HLA-extended haplotypes. The conditioning
regimen consisted of Bu 14 mg/kg followed by CY 200 mg/kg. GVHD proph
ylaxis was carried out with CsA plus short-term MTX. Allogeneic engraf
tment was obtained without any signs of acute or chronic GVHD, At fift
een months from the transplant the patient was in an excellent clinica
l condition. This case confirms the role of BMT from MUD in WAS, and s
uggests that complete donor-recipient identity for two entire HLA-exte
nded haplotypes is a particularly favorable immunogenetical condition
in this type of transplant.