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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

James L Harper
1   The Departments of Pediatrics and Internal Medicine, University of Nebraska College of Medicine, Omaha, NE, USA
,
Joan C Gill
2   The Great Lakes Hemophilia Foundation, Milwaukee, WI, USA
,
Russell J Hopp
1   The Departments of Pediatrics and Internal Medicine, University of Nebraska College of Medicine, Omaha, NE, USA
3   The Department of Pediatrics, Creighton University, Omaha, NE, USA
,
John Evans
4   The Department of Family Practice, St. Josephs Medical Center, Wichita, KS, USA
,
William D Haire
1   The Departments of Pediatrics and Internal Medicine, University of Nebraska College of Medicine, Omaha, NE, USA
› Author Affiliations
Further Information

Publication History

Received 11 April 1995

Accepted 29 June 1995

Publication Date:
09 July 2018 (online)

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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.

 

  • References

  • 1 Macik BG. Treatment of factor VIII inhibitors:products and strategies. Seminars in Thromb Hemost 1993; 19: 13-24
    PubMedGoogle Scholar
  • 2 Hay CRM, Lozier JN, Lee CA, Lafan M, Tradau H, Santagostino E, Civarella N, Schiavoni M, Fukui H, Yoshioka A, Teitel J, Mannucci PM, Kasper CK. Porcine factor VIII therapy in patients with congenital hemophilia and inhibitors: efficacy, patient selection, and side effects. Seminars in Hematology 1994; 31 (02) Suppl (Suppl. 04) 20-25
    PubMedGoogle Scholar
  • 3 Jamieson DM, Stafford CT, Maloney MJ, Lutcher CL. Desensitization to factor VIII in a patient with classical hemophilia and C2 deficiency. Annals of Allergy 1987; 58: 215-220
    PubMedGoogle Scholar
  • 4 Helmer RE, Alperin JB, Yunginger JW, Grant JA. Anaphylactic reactions following infusion of factor VIII in a patient with classical hemophilia. The American Journal of Medicine 1980; 69: 953-957
    CrossrefPubMedGoogle Scholar
  • 5 Maloney MJ, Stafford CT, Jamieson DM. Successful desensitization to factor VIII in a patient with classical hemophilia. Journal of Allergy and Clinical Immunology 1985; 75 210 Abst 421
    PubMedGoogle Scholar
  • 6 Nilsson IM. Immune Tolerance. Seminars in Hematology 1994; 31 (02) Suppl (Suppl. 04) 44-48
    PubMedGoogle Scholar