title: [The diagnosis and therapy of acquired factor-VIII inhibitors in combination with lupus anticoagulants].
abstract: METHODS
  A 63-year-old woman, known to have a primary factor VIII inhibitor (FFI) in combination with lupus anticoagulants (LA) was hospitalised because of life-threatening bleeding from mouth and neck.
  
  
  METHODS
  The activity of coagulation factor VIII was 9% under substitution, while the factor VIII inhibitor titre was 123 U/ml. A lupus anticoagulant test was positive. Antibodies against varicella-zoster virus and Epstein-Barr virus were demonstrated. The right adrenal was found to be enlarged on computed tomography.
  
  
  METHODS
  Coagulation became normal on administration of porcine factor VIII concentrate. Three cycles of a combination of three protein A immunoadsorptions, cyclophosphamide (twice 1.0 g intravenously), IgG (30 g daily for 5 days) as well as long-term oral cyclophosphamide administration (150 mg daily) during the interval were undertaken to reduce the inhibitor and produce immuno-tolerance. The factor VIII inhibitor titre was stabilised at a low level, but factor VIII activity could not be normalised without substitution.
  
  
  CONCLUSIONS
  The simultaneous presence of specific and non-specific inhibitors makes laboratory diagnosis and treatment more difficult. Porcine factor VIII and a combination of immunoadsorption and suppression are important components in the treatment of bleeding episodes and the production of immunotolerance.
authors.affiliation: Zentrallabor, Medizinische Universitätsklinik, Würzburg.
authors.forename: R
authors.initials: R
authors.lastname: Grossmann
authors.forename: H
authors.initials: H
authors.lastname: Wankmüller
authors.forename: S
authors.initials: S
authors.lastname: Schwender
authors.forename: J
authors.initials: J
authors.lastname: Kirschner
authors.forename: B
authors.initials: B
authors.lastname: Mansouri-Taleghani
authors.forename: P
authors.initials: P
authors.lastname: Keller
authors.forename: K
authors.initials: K
authors.lastname: Kochsiek
mesh_terms: D001327:Autoimmune Diseases / Q000097:blood / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D005169:Factor VIII / Q000032:analysis / Q000037:antagonists & inhibitors*; D005260:Female; D006470:Hemorrhage / Q000097:blood / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy; D006801:Humans; D007108:Immune Tolerance; D016682:Lupus Coagulation Inhibitor / Q000097:blood*; D008875:Middle Aged; D009059:Mouth Diseases / Q000097:blood / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy; D009333:Neck
chemical_list: D016682:Lupus Coagulation Inhibitor; D005169:Factor VIII
vernacular_title: Diagnose und Therapie eines erworbenen Faktor-VIII-Inhibitors in Kombination mit Lupus-Antikoagulanzien.