coagulation factor VIII autoantibody (acquired hemophilia)
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Etiology
- autoimmune diseases
- postpartum state
- hypersensitivity reactions to drugs
- low-grade lymphocytic malignancy
- may occur in older patients without known underlying disorder
Epidemiology
- postpartum: occurs most often after birth of 1st child
Clinical manifestations
- massive subcutaneous or intramuscular bleeding
- subdural hematoma[8]
- postpartum
- commonly 1-3 month delay prior to onset of symptoms
- bleeding tendency, bleeding gums
- variable course
Laboratory
- severely prolonged aPTT, NOT corrected with addition of normal plasma*
- coagulation factor VIII inhibitor in plasma
- prothrombin time is normal[8]
* an incomplete correction consistent with autoantibody (from 95 to 45 seconds)[5]
Differential diagnosis
Management
- immunosuppressive therapy
- acute bleeding
- recombinant factor VIIa concentrate
- acts to bypass need for factor VIII by binding to surface of activated platelets, where it can generate factor Xa[1]
- treatment of choice[1]
- prothrombin complex concentrate
- porcine factor VIII (generally less reactive with anti-human factor VIII)
- plasmapheresis
- recombinant factor VIIa concentrate
- low titers of autoantibody (< 5 Bethesda units, without acute bleeding) may be treated with factor VIII concentrate or desmopressin[1]
- immunosuppressive therapy
- high-dose oral corticosteroids with cyclophosphamide 2 mg/kg/day or rituximab to eradicate inhibitor[1]
- postpartum
- spontaneous resolution in most patients with 12-18 months of delivery
- recombinant factor VIIa[1]
- course shortened by immunosuppressive agents but not by corticosteroids alone
More general terms
Additional terms
- coagulation factor VIII alloantibody
- coagulation factor VIII inhibitor in plasma
- coagulation factor VIII; antihemophilic factor; AHF; procoagulant component; contains: factor VIIIa heavy chain, 200 kD & 92 isoforms; factor VIII B chain; factor VIIIa light chain (F8, F8C)
References
- ↑ Jump up to: 1.0 1.1 1.2 1.3 1.4 1.5 Medical Knowledge Self Assessment Program (MKSAP) 11, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2012, 2015. 2018, 2022.
- ↑ Schiller G, in: UCLA Intensive Course in Geriatric Medicine & Board Review, Marina Del Ray, CA, Sept 12-15, 2001
- ↑ Barnett B, Kruse-Jarres R, Leissinger CA. Current management of acquired factor VIII inhibitors. Curr Opin Hematol. 2008 Sep;15(5):451-5. PMID: https://www.ncbi.nlm.nih.gov/pubmed/18695367
- ↑ Gibson CJ, Berliner N, Miller AL et al A Bruising Loss. N Engl J Med 2016; 375:76-81. July 7, 201 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/27406351 <Internet> http://www.nejm.org/doi/full/10.1056/NEJMcps1500127
- ↑ Jump up to: 5.0 5.1 Geriatric Review Syllabus, 10th edition (GRS10) Harper GM, Lyons WL, Potter JF (eds) American Geriatrics Society, 2019
- ↑ Franchini M, Vaglio S, Marano G et al Acquired hemophilia A: a review of recent data and new therapeutic options. Hematology. 2017 Oct;22(9):514-520. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/28441921
- ↑ Kruse-Jarres R, Kempton CL, Baudo F er al Acquired hemophilia A: Updated review of evidence and treatment guidance. Am J Hematol. 2017 Jul;92(7):695-705. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/28470674 Free Article
- ↑ Jump up to: 8.0 8.1 8.2 8.3 NEJM Knowledge+ Hematology
Patient information
coagulation factor VIII autoantibody (acquired hemophilia) patient information