SUCCESSFUL TREATMENT OF A PATIENT WITH IDIOPATHIC FACTOR-VIII INHIBITOR WITH DOUBLE FILTRATION PLASMAPHERESIS AND STEROID-ADMINISTRATION

We report a 74-year-old Japanese woman who had bleeding due to a facto r VIII inhibitor in the absence of diseases known to be associated wit h its development. She abruptly developed a large painful purpura exte nding from the right hip to the thigh followed by an intramuscular hae matoma of her left anterior chest. Examinations revealed a marked depr ession of factor VIII activity (5%) and the presence of 31 Bethesda un its/ml factor VIII inhibiting activity. The phenotypes of these inhibi tors were of the IgG-kappa and IgG-lambda types. She was treated with oral prednisolone and double filtration plasmapheresis (DFPP). The inh ibitors rapidly disappeared after three sessions of plasmapheresis and her plasma factor VIII activity increased to a normal level. During t his treatment, no major adverse effects such as thrombosis and infecti on were observed and transfusion of fresh frozen plasma (FFP) was not necessary. Heterogeneity of idiopathic factor VIII inhibitors in elder ly patients is common and spontaneous disappearance or elimination of such inhibitors by treatment is often difficult to achieve. However, t he combination of oral prednisolone and double filtration plasmapheres is is effective and safe for idiopathic factor VIII inhibitors and is a worthwhile approach to treatment.