SUCCESSFUL TREATMENT OF TRANSIENT ACQUIRED FACTOR-X DEFICIENCY BY PLASMAPHERESIS WITH CONCOMITANT INTRAVENOUS IMMUNOGLOBULIN AND STEROID-THERAPY

Two patients with no history of previous bleeding diatheses presented with active bleeding from multiple body sites, declining hemoglobin le vels, and markedly prolonged prothrombin times (PT) and activated part ial thromboplastin times (aPTT) with incomplete correction on PT mix a ssays, Both patients demonstrated a severe deficiency of factor X (F.X ) (<1%; reference range 60-150%). F.X levels and bleeding were refract ory to multiple transfusions of fresh frozen plasma (FFP) in both pati ents, In contrast, daily therapeutic plasma exchange (PLEX) with conco mitant administration of intravenous immunoglobulin (IV IgG) and stero ids produced a rapid increase in F.X levels with cessation of bleeding , followed by stabilization and normalization of F.X levels and progre ssive correction of coagulation times, Neither patient has demonstrate d a recurrence of the bleeding tendency following discontinuation of s teroid therapy, These patients had transient acquired F.X deficiency, a rare coagulopathy, which can result in a lethal bleeding diathesis, An IgG inhibitor that selectively inhibited F.X activation in Russell' s viper venom or tissue factor/F.VIIa assays was demonstrated in one p atient's pretreatment plasma. Previous treatment of hemorrhage in tran sient acquired F.X deficiency has been prothrombin complex and/or acti vated clotting concentrates, which can be associated with transient hy percoagulable states. This is the first reported use of PLEX in transi ent acquired F.X deficiency, PLEX is safe, efficacious, and rapidly re stores hemostasis in this rare acquired bleeding disorder. (C) 1998 Wi ley-Liss, Inc.