title: [Post partum acquired hemophilia A with fatal outcome: a case report].
abstract: We report the case of a 19-year-old women who developed on post partum day 1 a large hematoma in the vaginal wall. A surgical intervention for evacuating the hematoma was complicated with profuse bleeding of the genital and abdominal cavities. Postoperative coagulation studies revealed a prolonged activated partial thromboplastin time (87/30 s; ratio: 2.9), a decreased factor VIII level at 7% and the presence of an anti-factor VIII inhibitor in a titer of 64 Bethesda Unit (BU). Due to the active bleeding, the patient received Novoseven(®) (recombinant factor VIIa: 2 doses on post partum day 3 and 1 dose on day 6) and Feiba(®) (activated prothrombin complex concentrates: 1 dose on post partum day 8). These few administrated doses were not enough efficacious to achieve a continuous control of the bleeding. Immunosuppressive treatment used prednisone alone for 3 days, then combined to cyclophosphamide. The outcome was marked by the aggravation of the bleeding and an increase in the inhibitor level (132 BU). Finally, the patient died on post partum day 8 following an hemorrhagic shock.
authors.affiliation: Laboratoire d'hématologie, Hôpital Habib Bourguiba, Sfax, Tunisie. mourad.chaari@yahoo.fr
authors.forename: Mourad
authors.initials: M
authors.lastname: Chaari
authors.forename: Riadh
authors.initials: R
authors.lastname: Bouhlel
authors.forename: Samy
authors.initials: S
authors.lastname: Kallel
authors.forename: Zied
authors.initials: Z
authors.lastname: Aïdi
authors.forename: Faiza
authors.initials: F
authors.lastname: Makni
authors.forename: Grigoris T
authors.initials: GT
authors.lastname: Gerotziafas
authors.forename: Ismail
authors.initials: I
authors.lastname: Elalamy
authors.forename: Abdelhamid
authors.initials: A
authors.lastname: Karoui
authors.forename: Choumous
authors.initials: C
authors.lastname: Kallel
mesh_terms: D000328:Adult; D001779:Blood Coagulation Factors / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D015942:Factor VIIa / Q000627:therapeutic use; D017809:Fatal Outcome; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006406:Hematoma / Q000188:drug therapy* / Q000209:etiology / Q000601:surgery; D006467:Hemophilia A / Q000188:drug therapy* / Q000276:immunology* / Q000601:surgery; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D006473:Postpartum Hemorrhage / Q000188:drug therapy* / Q000209:etiology / Q000601:surgery; D049590:Postpartum Period; D011247:Pregnancy; D011994:Recombinant Proteins / Q000627:therapeutic use; D014592:Uterine Hemorrhage / Q000188:drug therapy* / Q000209:etiology / Q000601:surgery
chemical_list: D001779:Blood Coagulation Factors; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D011994:Recombinant Proteins; D003520:Cyclophosphamide; C103587:recombinant FVIIa; C065655:anti-inhibitor coagulant complex; D015942:Factor VIIa