[Acquired haemophilia A: a rare but life-threatening auto-immune disorder].
A 31-year-old woman who was 5 months post partum, was presented to the Accident and Emergency Department because of acute abdominal pain and hypovolemic shock after being kicked in the stomach 3 days previously. She had a severe haemorrhage and a rupture of the liver. Attempts to selectively embolise the branches of the right hepatic artery failed and two laparotomies were performed. Analysis of the cause of the persistent liver bleeding and the prolonged APTT upon admission revealed the diagnosis acquired haemophilia A. The patient was treated with recombinant factor VIIa and a high dosage of corticosteroids. She made a good recovery after the corticosteroids had been replaced by rituximab.
Twiss
Eric
E
Canisius-Wilhelmina Ziekenhuis, afd. Heelkunde, Nijmegen, the Netherlands. ell_twiss@hotmail.com
Franschman
Gaby
G
Regelink
Josien
J
Zuidema
Wytse
W
D000328:Adult; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D001779:Blood Coagulation Factors / Q000627:therapeutic use; D015942:Factor VIIa / Q000627:therapeutic use*; D005260:Female; D006467:Hemophilia A / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D006470:Hemorrhage / Q000188:drug therapy* / Q000209:etiology* / Q000601:surgery; D006801:Humans; D011644:Puerperal Disorders; D016896:Treatment Outcome
D001779:Blood Coagulation Factors; D015942:Factor VIIa