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Antibody responses to bacteriophage phi X174 in patients with adenosine
deaminase deficiency
HD Ochs, RH Buckley, RH Kobayashi, AL Kobayashi, RU Sorensen, SD Douglas, BL Hamilton and MS Hershfield
Department of Pediatrics, University of Washington School of Medicine,
Seattle 98195.
Adenosine deaminase (ADA) deficiency and its biochemical consequences cause
severe combined immunodeficiency (SCID). Treatment strategies, designed to
correct the biochemical abnormalities, include transplantation of matched
bone marrow or haploidentical bone marrow stem cells, repeated partial
exchange transfusions with frozen irradiated human red blood cells (RBC),
or weekly injection of polyethylene glycol-modified bovine ADA (PEG-ADA).
To evaluate the effect of these therapeutic options, we studied in vitro
T-cell function and in vivo antibody responses to the T-cell-dependent
neoantigen, bacteriophage phi X174, in 10 children with ADA-deficient SCID.
In untreated patients, T-cell function was severely depressed, and only
minute amounts of antibacteriophage antibody were produced. Transplantation
of bone marrow from a matched sibling (one patient) or a phenotypically
matched parent (one patient) resulted in a stable graft, normal T-cell
function, and substantial but subnormal antibody titers to bacteriophage,
with reduced memory and impaired switch from IgM to IgG. Patients receiving
T-cell-depleted haploidentical bone marrow stem cells had markedly
depressed antibody responses for as long as 3 years posttransplantation,
despite rapidly improving T-cell function that became normal in two of four
patients. Two methods of enzyme replacement were explored. During treatment
with human RBC transfusions, antibody responses to bacteriophage were as
severely depressed as in untreated ADA-deficient patients. Treatment with
weekly injections of PEG-ADA resulted in normalization of T-cell numbers in
all four patients, normal or near-normal T-cell function in two, and mildly
but variably improved T-cell function in the other two patients.
Quantitatively and qualitatively normal antibody responses to bacteriophage
were observed in three of four patients. Assessment of antibody responses
to immunization with bacteriophage phi X174 is a useful method to monitor
humoral immune function in treated ADA- deficient patients and can be used
to estimate when intravenous immunoglobulin (IVIG) prophylaxis may be
safely discontinued.
Volume 80,
Issue 5,
pp. 1163-1171,
09/01/1992
Copyright © 1992 by The American Society of Hematology
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