Induction of Immune Tolerance in a 7-Year-Old Hemophiliac with an Anaphylactoid Inhibitor

James L Harper; Joan C Gill; Russell J Hopp; John Evans; William D Haire

doi:10.1055/s-0038-1649877

Thrombosis and Haemostasis, Table of Contents

Thromb Haemost 1995; 74(04): 1039-1041
DOI: 10.1055/s-0038-1649877

Original Article

Clinical Studies

Schattauer GmbH Stuttgart

Induction of Immune Tolerance in a 7-Year-Old Hemophiliac with an Anaphylactoid Inhibitor

Authors

James L Harper

¹The Departments of Pediatrics and Internal Medicine, University of Nebraska College of Medicine, Omaha, NE, USA
Joan C Gill

²The Great Lakes Hemophilia Foundation, Milwaukee, WI, USA
Russell J Hopp

¹The Departments of Pediatrics and Internal Medicine, University of Nebraska College of Medicine, Omaha, NE, USA

³The Department of Pediatrics, Creighton University, Omaha, NE, USA
John Evans

⁴The Department of Family Practice, St. Josephs Medical Center, Wichita, KS, USA
William D Haire

¹The Departments of Pediatrics and Internal Medicine, University of Nebraska College of Medicine, Omaha, NE, USA

Abstract

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Summary

Background: Anaphylactic reactions were a rare complication of low purity VIII concentrates, hut 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. KLISA revealed an anti-VIIl IgB 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 l00U/kg/d without symptoms. Results: His antibody titer decreased rapidly once he started l00U/kg/d. Six months later, the inhibitor was <1 Bethesda unit. Conclusion: Immune tolerance induction using a graduated dosage of rhVIII was successful.

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References

References
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