Open AccessAcquired haemophilia a presenting in postpartum period - case report
Author(s) -
Mirjana Mitrović-Vasiljević,
Darko Antić,
Ivo Elezović,
Jelena Bila,
Milena Todorović
Publication year - 2008
Publication title -
srpski arhiv za celokupno lekarstvo
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.135
H-Index - 17
eISSN - 2406-0895
pISSN - 0370-8179
DOI - 10.2298/sarh08s3214m
Subject(s) - medicine , cryoprecipitate , haemophilia a , haemophilia , vaginal delivery , pregnancy , surgery , postpartum period , complication , vaginal bleeding , fresh frozen plasma , recombinant factor viia , tranexamic acid , platelet , blood loss , biology , genetics
. 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. CASE OUTLINE. 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%. CONCLUSION. 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.