Acquired hemophilia A in a patient with systemic lupus erythematosus.
A patient with systemic lupus erythematosus (SLE) developed acquired hemophilia A. The patient, a 24-year-old Japanese woman, was referred to our hospital because of uncontrollable bleeding following a tooth extraction. Laboratory examination revealed prolonged APTT (116 seconds), reduced factor VIII activity (2.8 %) and the presence of factor VIII inhibitor at a titer of 46.5 Bethesda units/ml. Transfusion of prothrombin complex concentrate and activated prothrombin complex concentrate followed by administration of prednisolone and cyclophosphamide successfully arrested bleeding and reduced the factor VIII inhibitor level. Acquired hemophilia A is a rare but lethal condition. Rapid diagnosis and introduction of adequate therapies are critical.
Ishikawa
T
T
Third Department of Internal Medicine, Gunma University School of Medicine, Maebashi.
Tsukamoto
N
N
Suto
M
M
Uchiumi
H
H
Mitsuhashi
H
H
Yokohama
A
A
Maesawa
A
A
Nojima
Y
Y
Naruse
T
T
D000328:Adult; D005260:Female; D006467:Hemophilia A / Q000209:etiology*; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications*