Induction of immune tolerance in a 7-year-old hemophiliac with an anaphylactoid inhibitor

James Lloyd Harper, J. C. Gill, R. J. Hopp, J. Evans, W. D. Haire

Research output: Contribution to journalArticle

13 Citations (Scopus)

Abstract

Background: Anaphylactic reactions were a rare complication of low purity VIII concentrates, but not with; high purity VIII concentrates. Case: 7 y/o WM with severe hemophilia A, received only cryoprecipitate and monoclonally purified VIII concentrates; developed post-infusional urticaria. A 2-Bethesda-unit inhibitor was detected. Generalized urticaria and bronchospasm following factor developed as the titer increased. Skin tests demonstrated reactivity to plasma derived VIII, but not recombinant VIII (rhVIII). Attempts at desensitization using rhVIII failed. ELISA revealed an anti-VIII IgE antibody, He was treated with a modified tolerance regimen using rhVIII starting at 500 U/day with aggressive premedication. The dosage increased by 200 U weekly as tolerated to a maximum of 100 U/kg/d without symptoms. Results: His antibody titer decreased rapidly once he started 100 U/kg/d. Six months later, the inhibitor was <1 Bethesda unit. Conclusion: Immune tolerance induction using a graduated dosage of rhVIII was successful.

Original languageEnglish (US)
Pages (from-to)1039-1041
Number of pages3
JournalThrombosis and Haemostasis
Volume74
Issue number4
StatePublished - Jan 1 1995

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Immune Tolerance
Urticaria
Bronchial Spasm
Premedication
Hemophilia A
Anaphylaxis
Skin Tests
Enzyme-Linked Immunosorbent Assay
Antibodies
anti-IgE antibodies

ASJC Scopus subject areas

  • Hematology

Cite this

Induction of immune tolerance in a 7-year-old hemophiliac with an anaphylactoid inhibitor. / Harper, James Lloyd; Gill, J. C.; Hopp, R. J.; Evans, J.; Haire, W. D.

In: Thrombosis and Haemostasis, Vol. 74, No. 4, 01.01.1995, p. 1039-1041.

Research output: Contribution to journalArticle

Harper, JL, Gill, JC, Hopp, RJ, Evans, J & Haire, WD 1995, 'Induction of immune tolerance in a 7-year-old hemophiliac with an anaphylactoid inhibitor', Thrombosis and Haemostasis, vol. 74, no. 4, pp. 1039-1041.
Harper, James Lloyd ; Gill, J. C. ; Hopp, R. J. ; Evans, J. ; Haire, W. D. / Induction of immune tolerance in a 7-year-old hemophiliac with an anaphylactoid inhibitor. In: Thrombosis and Haemostasis. 1995 ; Vol. 74, No. 4. pp. 1039-1041.
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