hemophilia A; factor VIII deficiency

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Etiology

Epidemiology

  • occurs in men
  • 10-fold more common than hemophilia B
  • mean age of acquired form is 78 years of age
  • acquired form is rare

Genetics

Clinical manifestations

Laboratory

Complications

Management

*Previously, human factor VIII concentrate carried a high risk of transmitting HIV & hepatitis due to pooling of blood from large numbers of individuals. Since 1985, specific procedures to inactivate HIV & hepatitis C have been implemented for blood products obtained by pooling blood from large numbers of individuals. Recombinant factor VIII carries no risk of transmitting HIV or hepatitis.

More general terms

Additional terms

References

  1. DeGowin & DeGowin's Diagnostic Examination, 6th edition, RL DeGowin (ed), McGraw Hill, NY 1994, pg 893
  2. 2.0 2.1 2.2 2.3 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 16 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2012, 2015, 2018, 2022.
  3. 3.0 3.1 3.2 Collins PVV et al, Acquired hemophilia A in the United Kingdom: A 2-year national surveillance study by the United Kingdom. Haemophila Centre Doctors' Organisation Blood 2007, 109:1870 PMID: https://www.ncbi.nlm.nih.gov/pubmed/170047148
  4. 4.0 4.1 Leissinger C et al. Anti-inhibitor coagulant complex prophylaxis in hemophilia with inhibitors. N Engl J Med 2011 Nov 3; 365:1684 PMID: https://www.ncbi.nlm.nih.gov/pubmed/22047559
  5. 5.0 5.1 ARUP Consult: Hemophilia - Factor VIII or IX Deficiency The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/hemophilia
  6. 6.0 6.1 Broderick CR et al Association Between Physical Activity and Risk of Bleeding in Children With Hemophilia JAMA. 2012;308(14):1452-1459 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/23047359 <Internet> http://jama.jamanetwork.com/article.aspx?articleid=1377921
    Manco-Johnson MJ Collision Sports and Risk of Bleeding in Children With Hemophilia JAMA. 2012;308(14):1480-1481 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/23047364 <Internet> http://jama.jamanetwork.com/article.aspx?articleid=1377908
  7. Franchini M, Mannucci PM. Acquired haemophilia A: a 2013 update. Thromb Haemost. 2013 Dec;110(6):1114-20 PMID: https://www.ncbi.nlm.nih.gov/pubmed/24008306
  8. Sborov DW, Rodgers GM How I manage patients with acquired haemophilia A. Br J Haematol. 2013 Apr;161(2):157-65 PMID: https://www.ncbi.nlm.nih.gov/pubmed/23373521
  9. 9.0 9.1 9.2 Peyvandi F et al. A randomized trial of factor VIII and neutralizing antibodies in hemophilia A. N Engl J Med 2016 May 26; 374:2054 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/27223147 <Internet> http://www.nejm.org/doi/10.1056/NEJMoa1516437
    DiMichele DM. Hemophilia therapy - navigating speed bumps on the innovation highway. N Engl J Med 2016 May 26; 374:2087 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/27223151 <Internet> http://www.nejm.org/doi/10.1056/NEJMe1603419
  10. 10.0 10.1 Uchida N, Sambe T, Yoneyama K et al A first-in-human phase 1 study of ACE910, a novel factor VIII- mimetic bispecific antibody, in healthy subjects. Blood. 2016 Mar 31;127(13):1633-41. PMID: https://www.ncbi.nlm.nih.gov/pubmed/26626991 Free PMC Article
    Shima M et al. Factor VIII-mimetic function of humanized bispecific antibody in hemophilia A. N Engl J Med 2016 May 26; 374:2044. <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/27223146 <Internet> http://www.nejm.org/doi/10.1056/NEJMoa1511769
  11. 11.0 11.1 11.2 Anello J, Feinberg B, Heinegg J et al Hemophilia Guidelines on management of acute joint bleeds and chronic synovitis in hemophilia by the United Kingdom Haemophilia Centre Doctors' Organisation. Medscape: New Guidelines and Recommendations, August 2017. http://reference.medscape.com/viewarticle/884517
  12. 12.0 12.1 12.2 Oldenburg J, Mahlangu JN, Kim B et al Emicizumab Prophylaxis in Hemophilia A with Inhibitors. N Engl J Med 2017; 377:809-818. August 31, 2017 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/28691557 Free Article <Internet> http://www.nejm.org/doi/full/10.1056/NEJMoa1703068
  13. 13.0 13.1 Geriatric Review Syllabus, 11th edition (GRS11) Harper GM, Lyons WL, Potter JF (eds) American Geriatrics Society, 2022