title: Successful eradication of a FVIII inhibitor in a 60-year-old patient with mild haemophilia A using single-agent prednisolone.
abstract: BACKGROUND
  Development of FVIII inhibitors represents a major challenge in patients with mild haemophilia A (HA), because they tend to occur at an older age and classical immune tolerance induction appears to be less effective.
  
  
  METHODS
  A man (age: 60 years) with mild HA due to the missense mutation, Leu1929Arg, received a single dose of rFVIII at 35 IU/kg prior to routine colonoscopy, totalling 25 lifetime exposure days. Two months later, rFVIII was infused for a traumatic hip haematoma. However, FVIII recovery was inappropriate, and a FVIII inhibitor of 19 BU with type-2 kinetics was detected, resulting in FVIII:C of <1%. Two weeks later, the patient experienced spontaneous iliopsoas bleeding. Parallel to bypassing therapy, we started single-agent immunosuppression with prednisolone at 1.5mg/kg. FVIII:C "normalized" at 10.2% after four weeks. After five months, the inhibitor titre fell to <0.4 BU with sustained remission after one year of follow-up.
  
  
  CONCLUSIONS
  In mild HA, FVIII inhibitors may share characteristic features with FVIII autoantibodies commonly observed in acquired HA. Therefore, immunosuppressive therapy alone could be successful at least in a subset of patients.
authors.affiliation: II Medizinische Klinik und Poliklinik, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany. k.holstein@uke.de
authors.forename: K
authors.initials: K
authors.lastname: Holstein
authors.forename: G
authors.initials: G
authors.lastname: Marx
authors.forename: B
authors.initials: B
authors.lastname: Lentz
authors.forename: C
authors.initials: C
authors.lastname: Bokemeyer
authors.forename: F
authors.initials: F
authors.lastname: Langer
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D019774:Blood Coagulation Factor Inhibitors / Q000097:blood*; D005169:Factor VIII / Q000037:antagonists & inhibitors*; D006467:Hemophilia A / Q000097:blood* / Q000175:diagnosis / Q000188:drug therapy*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D019774:Blood Coagulation Factor Inhibitors; D005169:Factor VIII; D011239:Prednisolone