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Blood, Vol. 94 No. 8 (October 15), 1999:
pp. 2923-2930
Long-Term Chimerism and B-Cell Function After Bone Marrow
Transplantation in Patients With Severe Combined Immunodeficiency With
B Cells: A Single-Center Study of 22 Patients
Elie Haddad,
Françoise Le Deist,
Pierre Aucouturier,
Marina Cavazzana-Calvo,
Stephane Blanche,
Geneviève De Saint Basile, and
Alain Fischer
From the Unité d'Immunologie et d'Hématologie
Pédiatriques, Unité INSERM U429, and Laboratoire
d'Immunologie Clinique, INSERM U25, Hôpital Necker-Enfants
Malades, Paris, France.
We retrospectively analyzed the B-cell function and leukocyte
chimerism of 22 patients with severe combined immunodeficiency with B
cells (B+ SCID) who survived more than 2 years after bone
marrow transplantation (BMT) to determine the possible consequences of
BMT procedures, leukocyte chimerism, and SCID molecular deficit on
B-cell function outcome. Circulating T cells were of donor origin in
all patients. In recipients of HLA-identical BMT (n = 5), monocytes
were of host origin in 5 and B cells were of host origin in 4 and of
mixed origin in 1. In recipients of HLA haploidentical T-cell-depleted BMT (n = 17), B cells and monocytes were of host origin in 14 and of
donor origin in 3. Engraftment of B cells was found to be associated
with normal B-cell function. In contrast, 10 of 18 patients with host B
cells still require Ig substitution. Conditioning regimen (ie, 8 mg/kg
busulfan and 200 mg/kg cyclophosphamide) was shown neither to promote
B-cell and monocyte engraftment nor to affect B-cell function. Eight
patients with B cells of host origin had normal B-cell function.
Evidence for functional host B cells was further provided in 3 informative cases by Ig allotype determination and by the detection, in
5 studied cases, of host CD27+ memory B cells as in
age-matched controls. These results strongly suggest that, in some
transplanted patients, host B cells can cooperate with donor T cells to
fully mature in Ig-producing cells.
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