Recombinant human C1-inhibitor in the treatment of acute angioedema attacks

Goda Choi, Maarten R. Soeters, Henriette Farkas, Lilian Varga, Krystyna Obtulowicz, Barbara Bilo, Greg Porebski, C. Erik Hack, René Verdonk, Jan Nuijens, Marcel Levi

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Abstract

BACKGROUND: Patients with hereditary C1-inhibitor deficiency have recurrent attacks of angioedema, preferably treated with C1-inhibitor concentrate. A recombinant human C1-inhibitor (rHuC1INH) was developed, derived from milk from transgenic rabbits. This study was undertaken to investigate the effects of rHuC1INH in the treatment of acute angioedema attacks in patients with a hereditary C1-inhibitor deficiency. STUDY DESIGN AND METHODS: Patients with hereditary C1-inhibitor deficiency were treated with rHuC1INH (at a dose of 100 U/kg) within 8 hours after onset of an acute attack. Time to initiation of relief and time to minimal symptoms were reported by both the patient and the treating physician. RESULTS: Thirteen severe angioedema attacks in 9 patients with hereditary C1-inhibitor deficiency were treated with rHuC1INH. There was rapid improvement of clinical conditions for all attacks, with approximately 80 percent of treated patients experiencing symptom relief within 2 hours and minimal symptoms within 12 hours. There were no drug-related adverse events or immunogenic reactions to C1-inhibitor or rabbit proteins. CONCLUSION: The transgenically produced rHuC1INH was successfully used in the treatment of acute angioedema attacks in patients with hereditary C1-inhibitor deficiency.

Original languageEnglish
Pages (from-to)1028-1032
Number of pages5
JournalTransfusion
Volume47
Issue number6
DOIs
Publication statusPublished - Jun 1 2007

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ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology
  • Hematology

Cite this

Choi, G., Soeters, M. R., Farkas, H., Varga, L., Obtulowicz, K., Bilo, B., Porebski, G., Hack, C. E., Verdonk, R., Nuijens, J., & Levi, M. (2007). Recombinant human C1-inhibitor in the treatment of acute angioedema attacks. Transfusion, 47(6), 1028-1032. https://doi.org/10.1111/j.1537-2995.2007.01239.x