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Hemophilia, Acquired
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Structural domains of human factor VIII
Cell surface–directed hemostasis
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Acquired Hemophilia Resource Center

 
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Hemophilia, Acquired Hematology
  Acquired hemophilia is a rare but potentially life-threatening bleeding disorder caused by the development of autoantibodies directed against plasma coagulation factors, most frequently factor VIII (FVIII). Autoantibody inhibitors against factor II, factor V, factor VII, factor IX, factor X, factor XI, factor XIII, and von Willebrand factor proteins have also been reported. Diagnosis of acquired hemophilia can be difficult because of its rarity and because the patient does not have the usual precedent personal or family history of bleeding episodes seen in congenital hemophilia. Moreover, the clinical signs and symptoms of acquired hemophilia differ from those of hereditary hemophilia. Its severity at clinical presentation can also make managing acquired hemophilia challenging.
 
   
  CME

Management of Acquired Hemophilia in the Emergency Department

A 54-Year-Old Woman With Rheumatoid Arthritis, Bruising, Swelling, and Pain

Description
Barbara M, a 54-year-old white woman presents to the emergency department with complaints of fatigue, bruising, and increasing pain and swelling in the left posterior thigh, hip, and buttock. She denies any history of trauma, but she describes a tendency to easily bruise for the past 6 months.
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A 69-Year-Old Man With Prostate Cancer and Extensive Spontaneous Ecchymoses

Description
Anthony S, a 69-year-old white man with prostate cancer who is planning to undergo prostatectomy, presents to the emergency department (ED) with extensive spontaneous ecchymoses covering large areas of both arms, chest, right flank, and right thigh.
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  Clinical Trials
  Trial of Rituximab Versus Oral Cyclophosphamide to Eradicate or Suppress Autoimmune Anti-Factor VIII Antibodies in Patients With Acquired Hemophilia A
 
   
 
  Collins PW. Treatment of acquired hemophilia A J Thromb Haemost. 2007 May;5(5):893-900. Review.
  Franchini M. Acquired hemophilia A Hematology. 2006 Apr;11(2):119-25.
  Ma AD, Carrizosa D. Acquired Factor VIII Inhibitors: Pathophysiology and Treatment Hematology Am Soc Hematol Educ Program. 2006:432-7.
  Sumner MJ, Geldziler BD, Pedersen M, Seremetis S. Treatment of acquired haemophilia with recombinant activated FVII: a critical appraisal Haemophilia. 2007 Sep;13(5):451-61
  Franchini M. Rituximab in the treatment of adult acquired hemophilia A: A systematic review Crit Rev Oncol Hematol. 2007 Jul;63(1):47-52. Epub 2007 Jan 19. Review.
  Lambotte O, Dautremer J, Guillet B, Boutekedjiret T, Dreyfus M, Kotb R, Le Bras P, Delfraissy JF, Lambert T, Goujard C. Acquired hemophilia in older people: a poor prognosis despite intensive care J Am Geriatr Soc. 2007 Oct;55(10):1682-5.
  Brophy DF, Martin EJ, Nolte ME, Kuhn JG, Carr ME Jr. Effect of recombinant factor VIIa variant (NN1731) on platelet function, clot structure and force onset time in whole blood from healthy volunteers and haemophilia patients Haemophilia. 2007 Sep;13(5):533-41.
  Collins PW, Hirsch S, Baglin TP, Dolan G, Hanley J, Makris M, Keeling DM, Liesner R, Brown SA, Hay CR. Acquired haemophilia A in the UK: a two year national surveillance study by UK Haemophilia Centre Doctors' Organisation Blood. 2007 Mar 1;109(5):1870-7. Epub 2006 Oct 17.
  Bysted BV, Scharling B, Moller T, Hansen BL. A randomized, double-blind trial demonstrating bioequivalence of the current recombinant activated factor VII formulation and a new robust 25 degrees C stable formulation Haemophilia. 2007 Sep;13(5):527-32.
  Ng HJ, Tan DC, Lee LH. Treatment and outcome of acquired haemophilia A with a standard conventional regimen in a cohort without associated conditions Haemophilia. 2006 Jul;12(4):423-8.
  Berezne A, Stieltjes N, Le-Guern V, Teixeira L, Billy C, Roussel-Robert V, Flaujac C, Horellou MH, Guillevin L, Mouthon L. Rituximab alone or in association with corticosteroids in the treatment of acquired factor VIII inhibitors: report of two cases Transfus Med. 2006 Jun;16(3):209-12.
  Franchini M. Recombinant factor VIIa: a review on its clinical use Int J Hematol. 2006 Feb;83(2):126-38.
  Alvarado Y, Yao X, Jumper C, Hardwicke F, D'Cunha N, Cobos E. Acquired hemophilia: a case report of 2 patients with acquired factor VIII inhibotor treated with rituximab plus a short course of steroid and review of the literature Clin Appl Thromb Hemost. 2007 Oct;13(4):443-8.
  Brzoska M, Krause M, Geiger H, Betz C. Immunoadsorption with single-use columns for the management of bleeding in acquired hemophilia A: a series of nine cases J Clin Apher. 2007;22(4):233-40.



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