Acquired factor VIII:C inhibitor in a patient with Sjögren's syndrome: successful treatment with steroid and immunosuppressive therapy.
A 57-year-old woman affected with Sjögren's syndrome without bleeding history developed spontaneous hematomas at the arms, the left foot and the thigh, cutaneous hemorrhages and hematuria. Routine coagulation tests showed a prolongation of activated partial thromboplastin time associated with a marked reduction of factor VIII activity (VIII: C 5%). Other deficiencies of blood coagulation factors, especially von Willebrand factor, were excluded. Measurement of factor VIII inhibitor revealed an activity of 26.4 Bethesda units/ml. These findings were consistent with the diagnosis of acquired hemophilia A due to the presence of a factor VIII inhibitor. The patient was treated with a combination of prednisone and azathioprine. The therapy led, in a few months, to a significant reduction of factor VIII: C inhibitor and she did not require replacement therapy. Furthermore, there was a complete remission of the bleeding tendency. Long-term therapy for about 3 years induced the complete disappearance of the inhibitor and a full normalization of coagulation tests.
Dannhäuser
D
D
University of Padua, Medical School, Institute of Medical Semeiotics, Italy.
Casonato
A
A
Pietrogrande
F
F
Pontara
E
E
Bertomoro
A
A
Zerbinati
P
P
Girolami
A
A
D001132:Arm; D001323:Autoantibodies / Q000097:blood*; D001379:Azathioprine / Q000008:administration & dosage*; D003131:Combined Modality Therapy; D005169:Factor VIII / Q000032:analysis / Q000037:antagonists & inhibitors*; D005260:Female; D006406:Hematoma / Q000209:etiology / Q000276:immunology / Q000628:therapy; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D007165:Immunosuppression Therapy / Q000379:methods*; D007866:Leg; D008875:Middle Aged; D011241:Prednisone / Q000008:administration & dosage*; D012074:Remission Induction; D012859:Sjogren's Syndrome / Q000150:complications / Q000276:immunology* / Q000628:therapy*; D013997:Time Factors
D001323:Autoantibodies; D007074:Immunoglobulin G; D005169:Factor VIII; D001379:Azathioprine; D011241:Prednisone