title: Protein A sepharose immunoadsorption: immunological and haemostatic effects in two cases of acquired haemophilia.
abstract: Acquired haemophilia is a life-threatening disorder caused by circulating auto-antibodies that inhibit factor VIII coagulant activity (FBIII:C). Immunoadsorption on protein A sepharose (IA-PA) was performed in two bleeding patients with acquired haemophilia: we observed a dramatic and quick decrease in the anti-FVIII:C inhibitor titre leading to a normal, albeit transient, haemostatic status. In one case, IA-PA was the only procedure which succeeded in stopping massive haemorrhage. In the second case, IA-PA reinforced the haemostatic effect of recombinant activated factor VII by increasing the endogenous plasma factor VIII level. The efficacy of IA-PA was sustained with immunosuppressive treatment introduced, respectively, 10 and 15 d before the IA-PA procedures. Our experience with IA-PA suggests that this extracorporeal anti-FVIII:C removal procedure is a valuable therapeutic tool for acquired haemophilia and can alleviate life-threatening haemorrhages.
authors.affiliation: Laboratoire d'Hématologie et Centre de Référence et de Traitement de l'Hémophilie, Hôpital Bicêtre, AP-HP et Faculté de Médecine Paris XI, 94275 Le Kremlin-Bicêtre, Paris, France.
authors.forename: B
authors.initials: B
authors.lastname: Guillet
authors.forename: F
authors.initials: F
authors.lastname: Kriaa
authors.forename: M G
authors.initials: MG
authors.lastname: Huysse
authors.forename: V
authors.initials: V
authors.lastname: Proulle
authors.forename: C
authors.initials: C
authors.lastname: George
authors.forename: G
authors.initials: G
authors.lastname: Tchernia
authors.forename: R
authors.initials: R
authors.lastname: D'Oiron
authors.forename: Y
authors.initials: Y
authors.lastname: Laurian
authors.forename: B
authors.initials: B
authors.lastname: Charpentier
authors.forename: T
authors.initials: T
authors.lastname: Lambert
authors.forename: M
authors.initials: M
authors.lastname: Dreyfus
mesh_terms: D000328:Adult; D016063:Blood Loss, Surgical / Q000517:prevention & control; D015942:Factor VIIa / Q000627:therapeutic use; D006467:Hemophilia A / Q000276:immunology / Q000628:therapy*; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D007163:Immunosorbent Techniques; D008297:Male; D008875:Middle Aged; D010438:Peptic Ulcer Hemorrhage / Q000628:therapy; D010956:Plasmapheresis / Q000379:methods*; D011994:Recombinant Proteins / Q000627:therapeutic use; D013205:Staphylococcal Protein A
chemical_list: D007074:Immunoglobulin G; D011994:Recombinant Proteins; D013205:Staphylococcal Protein A; D015942:Factor VIIa