Rapid decrease in high titer of factor VIII inhibitors upon immunosuppressive treatment in severe postpartum acquired hemophilia A.
A 25-year-old woman was diagnosed with acquired hemophilia caused by factor VIII (FVIII) inhibitors two months after normal delivery of her first child. She presented with severe spontaneous bleeding in the right forearm and distal part of the right leg. After venipuncture for diagnostic purposes, severe hematoma developed, affecting the whole left arm and shoulder. The diagnosis of acquired hemophilia was confirmed by the presence of FVIII inhibitors (Bethesda units=76), low activity of factor VIII (5%), and prolonged activated partial thromboplastin time (APTT=89 s). The treatment with fresh frozen plasma, cryoprecipitate, red blood cells concentrate, methylprednisolone (3 mg/kg/day for 8 days, then 1 mg/kg/day), and cyclophosphamide (150 mg/day for 5 days, then 100 mg/day) was followed by the regression of hematomas and normalization of APTT and FVIII activity. The titer of FVIII inhibitors rapidly decreased and total disappearance of antibodies was achieved after 15 days. To the best of our knowledge, this is the shortest time to the disappearance of inhibitors to FVIII in postpartum acquired hemophilia ever reported. Transient reappearance of inhibitors occurred 10 months later and also promptly reacted to the same therapy. In the follow-up period of 4 years, the patient had two more pregnancies with no clinical and laboratory signs of illness recurrence. This case indicates important role of immunosuppressive therapy, which can rapidly induce a remission of this rare condition and be life-saving if introduced early in the course of illness and in doses higher than usually recommended.
Pejsa
Vlatko
V
Department of Internal Medicine, Dubrava University Hospital, Zagreb, Croatia. vpejsa@kbd.hr
Grgurević
Ivica
I
Kusec
Rajko
R
Gaćina
Petar
P
D000328:Adult; D001323:Autoantibodies / Q000097:blood; D003520:Cyclophosphamide / Q000627:therapeutic use*; D005169:Factor VIII / Q000037:antagonists & inhibitors* / Q000187:drug effects; D005260:Female; D006467:Hemophilia A / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D008775:Methylprednisolone / Q000627:therapeutic use*; D010314:Partial Thromboplastin Time; D006473:Postpartum Hemorrhage / Q000209:etiology*; D011247:Pregnancy; D013997:Time Factors
D001323:Autoantibodies; D003520:Cyclophosphamide; D005169:Factor VIII; D008775:Methylprednisolone