Spontaneous Antibodies against Factor VIII and Factor IX: A Case Report

Abstract

The common cause of isolated prolongation of aPTT is hemophilia or congenital deficiency of intrinsic factors especially factor VIII or IX activities which is recessive X-linked disorder. Therefore most cases of hemophilia are always men. If prolonged aPTT is found in women, spontaneous antibodies against any intrinsic clotting factor should be firstly considered as in our case. She is a 62-year old Thai woman who spontaneously develops large hematoma at left arm and right groin with low graded fever within one day. She has never had any episode of any excessive bleeding or family background. Coagulogram shows isolated prolonged aPTT that cannot be corrected in the mixed plasma. The activities of factor VIII and factor IX are markedly decreased. Therefore, the spontaneous or auto- antibodies directed against factor VIII and factor IX are diagnosed and they are treated with intravenous dexamethasone, fresh frozen plasma, factor VIII concentrate, packed red blood cells, danazol, azathioprine and antibiotics. She does not respond well, while the present hematomas gradually progress, a new hematoma appears at the right armpit. The aPTT cannot be normalized and she expires on the seventh day of admission. Besides various immunosuppressant as mentioned, the severe bleeding symptom in cases with factor VIII and IX auto-antibodies should have been additionally treated with the recombinant activated factor VII if it is available.