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Antibody responses to bacteriophage phi X174 in patients with adenosine deaminase deficiency.

Publication ,  Journal Article
Ochs, HD; Buckley, RH; Kobayashi, RH; Kobayashi, AL; Sorensen, RU; Douglas, SD; Hamilton, BL; Hershfield, MS
Published in: Blood
September 1, 1992

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.

Duke Scholars

Published In

Blood

ISSN

0006-4971

Publication Date

September 1, 1992

Volume

80

Issue

5

Start / End Page

1163 / 1171

Location

United States

Related Subject Headings

  • Severe Combined Immunodeficiency
  • Male
  • Immunology
  • Immunoglobulin G
  • Immunization
  • Humans
  • Female
  • Child, Preschool
  • Child
  • Bone Marrow Transplantation
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Ochs, H. D., Buckley, R. H., Kobayashi, R. H., Kobayashi, A. L., Sorensen, R. U., Douglas, S. D., … Hershfield, M. S. (1992). Antibody responses to bacteriophage phi X174 in patients with adenosine deaminase deficiency. Blood, 80(5), 1163–1171.
Ochs, H. D., R. H. Buckley, R. H. Kobayashi, A. L. Kobayashi, R. U. Sorensen, S. D. Douglas, B. L. Hamilton, and M. S. Hershfield. “Antibody responses to bacteriophage phi X174 in patients with adenosine deaminase deficiency.Blood 80, no. 5 (September 1, 1992): 1163–71.
Ochs HD, Buckley RH, Kobayashi RH, Kobayashi AL, Sorensen RU, Douglas SD, et al. Antibody responses to bacteriophage phi X174 in patients with adenosine deaminase deficiency. Blood. 1992 Sep 1;80(5):1163–71.
Ochs, H. D., et al. “Antibody responses to bacteriophage phi X174 in patients with adenosine deaminase deficiency.Blood, vol. 80, no. 5, Sept. 1992, pp. 1163–71.
Ochs HD, Buckley RH, Kobayashi RH, Kobayashi AL, Sorensen RU, Douglas SD, Hamilton BL, Hershfield MS. Antibody responses to bacteriophage phi X174 in patients with adenosine deaminase deficiency. Blood. 1992 Sep 1;80(5):1163–1171.

Published In

Blood

ISSN

0006-4971

Publication Date

September 1, 1992

Volume

80

Issue

5

Start / End Page

1163 / 1171

Location

United States

Related Subject Headings

  • Severe Combined Immunodeficiency
  • Male
  • Immunology
  • Immunoglobulin G
  • Immunization
  • Humans
  • Female
  • Child, Preschool
  • Child
  • Bone Marrow Transplantation