SpringerLink
Log in

Approach to a Child with Hemophilia B and Anaphylaxis to Factor IX

  • Chapter
  • First Online:
Pediatric Bleeding Disorders

  • 466 Accesses

Abstract

The development of inhibitory antibodies is the most challenging complication in the management of patients with hemophilia. Although much rarer than the occurrence of inhibitors in hemophilia A, the development of inhibitors in patients with hemophilia B can be complicated by anaphylactoid reactions. Immune tolerance therapy in patients with hemophilia B has a lower success rate than in patients with hemophilia B and can lead to the development of steroid-resistant nephrotic syndrome.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or Ebook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 54.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 69.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free ship** worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Similar content being viewed by others

References

  1. Miller CH, Platt SJ, Rice AS, Kelly F, Soucie JM. Validation of Nijmegen-Bethesda assay modifications to allow inhibitor measurement during replacement therapy and facilitate inhibitor surveillance. J Thromb Haemost. 2012;10(6):1055–61.

    Article  CAS  Google Scholar 

  2. Barnes C, Davis A, Furmedge J, Egan B, Donnan L, Monagle P. Induction of immune tolerance using rituximab in a child with severe haemophilia B with inhibitors and anaphylaxis to factor IX. Haemophilia. 2010;16(5):840–1.

    Article  CAS  Google Scholar 

  3. Darby SC, Keeling DM, Spooner RJD, Wan Kan S, Giangrande PLF, Collins PW, Hill FGH, Hay CRM. The incidence of factor VIII and factor IX inhibitors in the hemophilia population of the UK and their effect on subsequent mortality, 1977-99. J Thromb Haemost. 2004;2(7):1047–54.

    Article  CAS  Google Scholar 

  4. Ljung R, Petrini P, Tengborn L, Sjorin E. Haemophilia B mutations in Sweden: a population-based study of mutational heterogeneity. Br J Haematol. 2001;113(1):81–6.

    Article  CAS  Google Scholar 

  5. Puetz J, Soucie JM, Kempton CL, Monahan PE. Prevalent inhibitors in haemophilia B subjects enrolled in the Universal Data Collection database. Haemophilia. 2014;20(1):25–31.

    Article  CAS  Google Scholar 

  6. Saini S, Hamasaki-Katagiri N, Pandey GS, Yanover C, Guelcher C, Simhadri VL, Dandekar S, Guerrera MF, Kimchi-Sarfaty C, Sauna ZE. Genetic determinants of immunogenicity to factor IX during the treatment of haemophilia B. Haemophilia. 2015;21(2):210–8.

    Article  CAS  Google Scholar 

  7. Chitlur M, Warrier I, Rajpurkar M, Lusher JM. Inhibitors in factor IX deficiency a report of the ISTH-SSC international FIX inhibitor registry (1997-2006). Haemophilia. 2009;15(5):1027–31.

    Article  CAS  Google Scholar 

  8. Hay CR, DiMichele DM. The principal results of the International Immune Tolerance Study: a randomized dose comparison. Blood. 2012;119(6):1335–44.

    Article  CAS  Google Scholar 

  9. DiMichele DM, Kroner BL. Analysis of the North American Immune Tolerance Registry (NAITR) 1993–1997: current practice implications. Vox Sang. 1999;77(Suppl. 1):31–2.

    Article  CAS  Google Scholar 

  10. Dioun AF, Ewenstein BM, Geha RS, Schneider LC. IgE-mediated allergy and desensitization to factor IX in hemophilia B. J Allergy Clin Immunol. 1998;102(1):113–7.

    Article  CAS  Google Scholar 

  11. Shibata M, Shima M, Misu H, Okimoto Y, Giddings JC, Yoshioka A. Management of haemophilia B inhibitor patients with anaphylactic reactions to FIX concentrates. Haemophilia. 2003;9(3):269–71.

    Article  CAS  Google Scholar 

  12. Mathias M, Khair K, Hann I, Liesner R. Rituximab in the treatment of alloimmune factor VIII and IX antibodies in two children with severe haemophilia. Br J Haematol. 2004;125(3):366–8.

    Article  Google Scholar 

  13. Fox RA, Neufeld EJ, Bennett CM. Rituximab for adolescents with haemophilia and high titre inhibitors. Haemophilia. 2006;12(3):218–22.

    Article  CAS  Google Scholar 

  14. Curry NS, Misbah SA, Giangrande PLF, Keeling DM. Achievement of immune tolerance in a patient with haemophilia B and inhibitory antibodies, complicated by an anaphylactoid reaction. Haemophilia. 2007;13(3):328–30.

    Article  CAS  Google Scholar 

  15. Alexander S, Hopewell S, Hunter S, Chouksey A. Rituximab and desensitization for a patient with severe factor IX deficiency, inhibitors, and history of anaphylaxis. J Pediatr Hematol Oncol. 2008;30(1):93–5.

    Article  Google Scholar 

  16. Chuansumrit A, Moonsup Y, Sirachainan N, Benjaponpitak S, Suebsangad A, Wongwerawattanakoon P. The use of rituximab as an adjuvant for immune tolerance therapy in a hemophilia B boy with inhibitor and anaphylaxis to factor IX concentrate. Blood Coagul Fibrinolysis. 2008;19(3):208–11.

    Article  CAS  Google Scholar 

  17. Klarmann D, Martinez Saguer I, Funk MB, Knoefler R, von Hentig N, Heller C, Kreuz W. Immune tolerance induction with mycophenolate-mofetil in two children with haemophilia B and inhibitor. Haemophilia. 2008;14(1):44–9.

    Article  CAS  Google Scholar 

  18. Beutel K, Hauch H, Rischewski J, Kordes U, Schneppenheim J, Schneppenheim R. ITI with high-dose FIX and combined immunosuppressive therapy in a patient with severe haemophilia B and inhibitor. Hamostaseologie. 2009;29(2):155–7.

    Article  CAS  Google Scholar 

  19. Barnes C, Davis A, Furmedge J, Egan B, Donnan L, Monagle P. Induction of immune tolerance using rituximab in a child with severe haemophilia B with inhibitors and anaphylaxis to factor IX. Haemophilia. 2010;16(5):840–1.

    Article  CAS  Google Scholar 

  20. Holstein K, Schneppenheim R, Schrum J, Bokemeyer C, Langer F. Successful second ITI with factor IX and combined immunosuppressive therapy. Hamostaseologie. 2017;34(Suppl 1):S5–8.

    Google Scholar 

  21. Bon A, Morfini M, Dini A, Mori F, Barni S, Gianluca S, de Martino M, Novembre E. Desensitization and immune tolerance induction in children with severe factor IX deficiency; inhibitors and adverse reactions to replacement therapy: a case-report and literature review. Ital J Pediatr. 2015;41:12.

    Article  Google Scholar 

  22. Dharnidharka VR, Takemoto C, Ewenstein BM, Seymour R, William Harris H. Membranous glomerulonephritis and nephrosis post factor IX infusions in hemophilia B. Pediatr Nephrol. 1998;12(8):654–7.

    Article  CAS  Google Scholar 

  23. Ewenstein BM, Takemoto C, Warrier I, Lusher J, Saidi P, Eisele J, Ettinger LJ, DiMichele D. Nephrotic Syndrome as a Complication of Immune Tolerance in Hemophilia B. Blood. 1997;89(3):1115–5.

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pediatrics, St. Louis Children’s Hospital, St. Louis, MO, USA

    Surbhi Saini

  2. Washington University in St. Louis School of Medicine, St. Louis, MO, USA

    Surbhi Saini

  3. Nationwide Children’s Hospital, Division of Pediatric Hematology, Oncology and Bone Marrow Transplant, Columbus, OH, USA

    Amy L. Dunn

  4. Department of Pediatrics, The Ohio State University College of Medicine, Columbus, OH, USA

    Amy L. Dunn

Authors
  1. Surbhi Saini
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Amy L. Dunn
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Amy L. Dunn .

Editor information

Editors and Affiliations

  1. Division of Pediatric Hematology, Oncology and Bone Marrow Transplant, Nationwide Children’s Hospital, Columbus, OH, USA

    Amy L. Dunn

  2. Division of Pediatric Hematology, Oncology and Bone Marrow Transplant, Nationwide Children’s Hospital, Columbus, OH, USA

    Bryce A. Kerlin

  3. Division of Pediatric Hematology, Oncology and Bone Marrow Transplant, Nationwide Children’s Hospital, Columbus, OH, USA

    Sarah H. O'Brien

  4. Division of Pediatric Hematology, Oncology and Bone Marrow Transplant, Nationwide Children’s Hospital, Columbus, OH, USA

    Melissa J. Rose

  5. Division of Pediatric Hematology, Oncology and Bone Marrow Transplant, Nationwide Children’s Hospital, Columbus, OH, USA

    Riten Kumar

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Saini, S., Dunn, A.L. (2020). Approach to a Child with Hemophilia B and Anaphylaxis to Factor IX. In: Dunn, A., Kerlin, B., O'Brien, S., Rose, M., Kumar, R. (eds) Pediatric Bleeding Disorders. Springer, Cham. https://doi.org/10.1007/978-3-030-31661-7_4

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-31661-7_4

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-31660-0

  • Online ISBN: 978-3-030-31661-7

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation