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ABSTRACT An 18 month old boy with Wiskott-Aldrich Syndrome (WAS) received a marrow transplant from an HLA-matched sister after being conditioned with cyclophosphamide and dimethyl-myleran;
post transplant methotrexate was given for 6 weeks. Complete hematologic reconstitution with donor cells occurred. In vitro immunoglobulin (Ig) synthesis was determined with a direct and an
indirect hemolytic plaque assay using pokeweed mitogen activation and co-culture techniques. Before transplantation, Ig secretion of B cells was impaired; T-cells provided adequate help and
suppressor T-cells were not detected. Following transplantation, a transient impairment of IgG secretion by patient's B-cells in the presence of autologous or normal allogeneic T-cells
was observed. During three months of post-transplant study, T-helper activity was persistently defective and Ig secretion by normal B and T cells was suppressed (> 95%) by the
patient's T cells. Recovery from the transplant procedure was rapid and 3 months after the transplant the patient was infection-free and without signs of graft versus host disease;
serum immunoglobulin concentrations were normal and isohemagglutinins became demonstrable; he responded to bacteriophage ØX 174 with adequate antibody titers and in vitro lymphocyte
transformation to mitogens was 16-51% of normal. Presently, eight months after the transplant, he is asymptomatic and thriving. These observations indicate that hematologic and immunologic
reconstitution can be achieved in WAS without total body irradiation prior to marrow infusion, and that the appearance of donor derived suppressor T cells may play a role in the homeostasis
between the host and graft. ARTICLE PDF AUTHOR INFORMATION AUTHORS AND AFFILIATIONS * Departments of Pediatrics and Medicine, University of Washington, Seattle Lawrence G Lum, Hans D Ochs, F
Leonard Johnson & Rainer F Storb Authors * Lawrence G Lum View author publications You can also search for this author inPubMed Google Scholar * Hans D Ochs View author publications You
can also search for this author inPubMed Google Scholar * F Leonard Johnson View author publications You can also search for this author inPubMed Google Scholar * Rainer F Storb View author
publications You can also search for this author inPubMed Google Scholar RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE Lum, L., Ochs, H., Johnson, F.
_et al._ 831 WISKOTT-ALDRICH SYNDROME: IMMUNE STATUS AFTER BONE MARROW TRANSPLANTATION. _Pediatr Res_ 15 (Suppl 4), 581 (1981). https://doi.org/10.1203/00006450-198104001-00856 Download
citation * Issue Date: 01 April 1981 * DOI: https://doi.org/10.1203/00006450-198104001-00856 SHARE THIS ARTICLE Anyone you share the following link with will be able to read this content:
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