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Immune reconstitution in severe combined immunodeficiency disease after
lectin-treated, T-cell-depleted haplocompatible bone marrow transplantation
Y Dror, R Gallagher, DW Wara, BW Colombe, A Merino, M Benkerrou and MJ Cowan
Division of Pediatric Bone Marrow Transplant and Pediatric Immunology,
University of California, San Francisco 94143.
We describe our 9-year experience with lectin-treated T-cell-depleted
haplocompatible parental bone marrow transplantation (BMT) for 24 patients
with severe combined immunodeficiency disease (SCID). Nineteen of 21
evaluable patients had T-cell engraftment; 2 of 11 patients tested had
B-cell and monocyte engraftment. Fourteen of 24 (58%) patients are alive 7
months to 9.8 years post-BMT. Seventeen of 24 patients received
pretransplant conditioning with chemotherapy and/or total body irradiation,
and 8 of 24 received more than one transplant. Patients who received
conditioning had a survival rate of 61% versus 57% for those who received
no conditioning. None received graft-versus- host disease (GVHD)
prophylaxis and no patient had acute or chronic GVHD greater than grade I.
Kinetics and follow-up of immune recovery were analyzed in 14 patients who
are greater than 1 year from transplant. Half of the patients showed
evidence of T-cell function by 3 months and normal T-cell function by 4 to
7 months post-BMT. On average, T-cell numbers and subsets became normal 10
to 12 months posttransplant. Recovery of B-cell function was more delayed,
although in most patients B-cell numbers and IgM levels were normal by 12
months post-BMT. B-cell function, as determined by isohemagglutinin titers
or specific antibodies to pneumococcal polysaccharide, keyhole limpet
hemocyanin, or tetanus toxoid, became normal in 10 of 14 patients 2 to 8
years post-BMT. Seven of the 14 are off gammaglobulin therapy. Production
of isohemagglutinins tended to predict recovery of antibody response to
pneumococcal polysaccharide (P < .064). Based on these results, we
believe that haplocompatible BMT is an effective, curative treatment for
patients with SCID who lack an HLA-matched related donor.
Volume 81,
Issue 8,
pp. 2021-2030,
04/15/1993
Copyright © 1993 by The American Society of Hematology
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