[Acquired haemophilia A presenting in postpartum period--case report].
BACKGROUND
Acquired haemophilia A is a rare autoimmune disorder caused by factor VIII (FVIII) inhibitors. Patients may present with catastrophic bleeding, despite having no prior history of bleeding disorders. Acquired haemophilia A is a rare complication of pregnancy, typically appearing in the postpartum period. The patients usually present with bleeding related to vaginal delivery or Caesarean section. Management includes control of haemorrhage and eradication of the FVIII inhibitor.


METHODS
Acquired haemophilia A was diagnosed in our patient after profuse vaginal bleeding related to vaginal delivery (FVIII level 2%; FVIII inhibitor titar 16 BJ). Red cell, fresh frozen plasma and cryoprecipitate transfusions, as well as vaginal packing were ineffective. The administration of recombinant activated factor VII (rFVIIa) (NovoSeven) proved effective in stopping the bleeding. To facilitate eradication of the inhibitor prednisone (30 mg orally/d) was added. After 3 weeks of treatment, the inhibitor FVIII titar was reduced to 50%.


CONCLUSIONS
Acquired postpartum haemophilia must be always considered in the differential diagnosis of postpartum haemorrhage. According to our experience and reported literature (case reports and small series), initial haemodynamic stabilization after rFVIIa (NovoSeven) administration followed by immunosuppressive therapy is highly successful. Thus, most women presenting with acguired hemophilia A in the portpartum period has favourable prognosis.
Mitrović-Vasiljević
Mirjana
M
Antić
Darko
D
Elezović
Ivo
I
Bila
Jelena
J
Todorović
Milena
M
D000328:Adult; D015942:Factor VIIa / Q000627:therapeutic use*; D005260:Female; D006467:Hemophilia A / Q000097:blood / Q000150:complications*; D006490:Hemostatics / Q000627:therapeutic use*; D006801:Humans; D006473:Postpartum Hemorrhage / Q000188:drug therapy* / Q000209:etiology; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic; D011994:Recombinant Proteins / Q000627:therapeutic use; D055815:Young Adult
D006490:Hemostatics; D011994:Recombinant Proteins; C103587:recombinant FVIIa; D015942:Factor VIIa