Volume 4 Supplement 3

6th Drug Hypersensitivity Meeting (DHM 6)

Open Access

Successful desensitization in a patient with Von Willebrand´s disease and anaphylaxis to factor VIII/VW

  • Maristela Olival1,
  • Mara Felix2,
  • Monica Soares2,
  • Marilia Renni1,
  • Silmara Montalvao3,
  • Luis Ensina4 and
  • Mariana Castells5
Clinical and Translational Allergy20144(Suppl 3):P61

https://doi.org/10.1186/2045-7022-4-S3-P61

Published: 18 July 2014

Background

Von Willebrand´s disease (VWD) is the most common congenital disorder of hemostasis, characterized by deficient or defective von Willebrand factor. Patients are treated by intravenous replacement of factor VIII/VW (FVIII/VW) when needed, for prophylaxis before surgical procedures. Anaphylactic reactions to FVIII/VW are rare and desmopressin (DDAVP) can be used as an alternative.

Materials and methods

We report a case of a 16-year-old female, with VWD and no personal or family history of atopic disease, that was referred to our unit after 4 episodes of facial, upper and lower extremities urticaria, 30 minutes after infusion of FVIII/VW. She has been treated with the factor since 2000, and had her first allergic reaction in 2004. After the first episode, she received pretreatment with oral dexchlorpheniramine and hydrocortisone IV. The last episode was in March 2013, when she had laryngeal edema, which required epinephrine. The factor was replaced by DDAVP, but she developed itching, flushing and hypertension. We evaluated the patient using the ENDA questionnaire and skin tests. Skin prick test (SPT) to FVIII/VW (Octavi® SDOptimum; Octapharma, Vienna, Austria) was performed at full-strength concentration (50 U/ml) with histamine as positive control and normal saline as negative control. Intradermal skin tests (ID) were performed with 1:1000, 1:100, 1:10 and 1:1 concentrations. The reactions were assessed at 15 minutes. Her mother served as control subject and gave informed consent before testing and desensitization. A desensitization protocol was designed for the patient. Briefly, intravenous infusions of FVIII/FVW were started at 0.01 U/kg and doses were doubled at 10-minute intervals initially and then at 15-minute intervals to reach a cumulative dose of 30 U/ml. She was pre-medicated with diphenhydramine 25 mg, ranitidine 50 mg and montelukast 10 mg.

Results

SPT were negative in both patient and control subject. ID reactions at all dilutions were negative in control subject but it was positive at 1:1000 dilution in the patient (wheal of 8 mm). She developed an urticarial rash 4 hours after skin tests, treated with fexofenadine. Four days later, she underwent successful desensitization which was completed in 2 hours and 35 minutes.

Conclusions

Rapid desensitization for FVIII/VW induced anaphylaxis should be considered in patients with VWD to allow for safe surgical procedures.

Authors’ Affiliations

(1)
Department of Hematology, Clinical Hematology Divi, Hematological State Institute “Arthur de Siqueira Cavalcanti”– HEMORIO, Brazil
(2)
Department of Pediatrics, Allergy and Immunology, Federal Hospital of Servidores do Estado
(3)
University of Campinas, Hematology and Hemotherapy Center
(4)
Federal University of São Paulo, Division of Clinical Immunology and Allergy
(5)
Department of Medicine, Division of Rheumatology, Brigham and Women’s Hospital

Copyright

© Olival et al; licensee BioMed Central Ltd. 2014

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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