A 66-year-old non-hemophiliac man developed widely spreaded
hematoma in soft tissues around neck. On the day of admission, he was nearly choked by the giant
hematoma which markedly compressed trachea. Initially, to keep air way, an intratracheal intubation using a fiber scope was carried out. Coagulation studies revealed a marked prolongation of activated partial thromboplastin time (129 seconds, control: 24.9-33.2), a diminished activity of
factor VIII (less than 1%) and a significant increase of
factor VIII inhibitor titer (60 Bethesda unit/ml). We thereby diagnosed coagulopathy related to acquired inhibitor against
factor VIII. The general combination
therapy of
intravenous infusion of both
prothrombin complex concentrates and activated
prothrombin complex concentrates,
prednisolone and
plasma exchange successfully stopped expansion of the giant
hematoma. However, because the titer of
factor VIII inhibitor remained increased and the activity of
factor VIII was continued to reduced,
cyclosporin was additionally administrated. As a result, the titer of
factor VIII inhibitor was markedly improved, but the reduced activity of
factor VIII was continued and re-
bleeding around neck was observed. We thereby administered
steroid pulse
therapy, which resulted
in complete remission of the disease activity and recovery of
factor VIII activity.