Successful treatment of transient acquired factor X deficiency by plasmapheresis with concomitant intravenous immunoglobulin and steroid therapy

Abstract

Two patients with no history of previous bleeding diatheses presented with active bleeding from multiple body sites, declining hemoglobin levels, and markedly prolonged prothrombin times (PT) and activated partial thromboplastin times (aPTT) with incomplete correction on PT mix assays. Both patients demonstrated a severe deficiency of factor X (F.X) (<1%; reference range 60-150%). F.X levels and bleeding were refractory to multiple transfusions of fresh frozen plasma (FFP) in both patients. In contrast, daily therapeutic plasma exchange (PLEX) with concomitant administration of intravenous immunoglobulin (IV IgG) and steroids produced a rapid increase in F.X levels with cessation of bleeding, followed by stabilization and normalization of F.X levels and progressive correction of coagulation times. Neither patient has demonstrated a recurrence of the bleeding tendency following discontinuation of steroid 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 patient's pretreatment plasma. Previous treatment of hemorrhage in transient acquired F.X deficiency has been prothrombin complex and/or activated clotting concentrates, which can be associated with transient hypercoagulable states. This is the first reported use of PLEX in transient acquired F.X deficiency. PLEX is safe, efficacious, and rapidly restores hemostasis in this rare acquired bleeding disorder.

Treatment of acquired factor X inhibitor by plasma exchange with concomitant intravenous immunoglobulin and corticosteroids

“…Acquired factor X deficiency is even rarer and is associated with amyloidosis in the majority of cases [3]. Only a few cases of factor X deficiency attributed to an inhibitor in adults have been described in the literature [4][5][6][7][8][9]. The inhibitor may be a specific factor X inhibitor [4][5][6][7][8] or a nonspecific lupus anticoagulant [9].…”

“…Clinically, patients with an acquired factor X inhibitor present with a wide range of hemorrhagic manifestations including mucocutaneous and deep tissue bleeding [4][5][6][7]. Typically, these patients do not have a prior history of bleeding, toxin exposure, liver or autoimmune disease [4][5][6][7].…”

“…Typically, these patients do not have a prior history of bleeding, toxin exposure, liver or autoimmune disease [4][5][6][7]. Treatment with fresh frozen plasma (FFP) and vitamin K are, in general, unsuccessful [4,5,7].…”

“…Typically, these patients do not have a prior history of bleeding, toxin exposure, liver or autoimmune disease [4][5][6][7]. Treatment with fresh frozen plasma (FFP) and vitamin K are, in general, unsuccessful [4,5,7]. A few anecdotal reports of successful treatments using various therapies have been described [4][5][6][7][8].…”

“…Treatment with fresh frozen plasma (FFP) and vitamin K are, in general, unsuccessful [4,5,7]. A few anecdotal reports of successful treatments using various therapies have been described [4][5][6][7][8].…”