V. Brunel et al., DIRECT EVIDENCE FOR DISSOCIATED MEGAKARYOCYTIC CHIMERISM IN A WISKOTT-ALDRICH PATIENT SUCCESSFULLY ALLOGRAFTED, British Journal of Haematology, 90(2), 1995, pp. 336-340
We report a Wiskott-Aldrich patient who underwent allogeneic bone marr
ow transplantation from his HLA-identical sister at the age of 25. Con
ditioning regimen consisted of cyclophosphamide (180mg/kg) and thoraco
-abdominal irradiation (6 Grays). Cytogenetic follow-up revealed rapid
and complete lymphoid chimaerism, but prolonged mixed bone marrow chi
maerism. Correlative interphase cytogenetics performed on bone marrow
smears using dual-colour fluorescence in situ hybridization with X and
Y specific probes showed that the proportion of donor cells was signi
ficantly higher within megakaryocytes than in other lineages, This pat
ient therefore presented with dissociated lineage engraftment, which i
s not exceptional in congenital diseases and aplastic anaemia, but has
not previously been described in Wiskott-Aldrich syndrome. Bone marro
w transplantation was successful despite this delayed engraftment whic
h ensured adequate production in the involved cell lines.