A 68 year-old house-wife, who had neither past nor family history of hemorrhagic diathesis, developed spontaneous bleeding of the palate in February, 1983, followed by swelling and pain in her ankle joints. She was hospitalized for this hemorrhagic diathesis in June, 1983. The laboratory data were as follows: activated partial thromboplastin time (APTT), 75 seconds; factor VIII procoaglant activity, 0%; factor VIII related antigen, 160%; von Willebrand's factor, 88%. The level of factor VIII inhibitor was remarkably high, 250 Bethesda units. A diagnosis of bleeding due to the presence of the factor VIII inhibitor was made. The inhibitor was an immunoglobulin of IgG type, which had both K and L light chain. Only 24 cases of hemorrhagic diathesis caused by spontaneous factor VIII inhibitor have been reported sofar, including this case, in Japan.
Bleeding of non-hemophilic patients with factor VIII inhibitor have usually been treated with corticosteroids, immunosuppresive agents, plasmapheresis and so on. These treatments were tried with no noticeable effect in our case. Therefore, prothrombin complex concentrages (PCC), Autoplex and Proplex, were administered to stop the bleeding. This resulted in the improvement of these symptoms, as shown by the TEG, APTT and PT values. It appears that PCC are effective in the treatment of bleeding due to a high spontaneous factor VIII inhibitor level.
