[Acquired haemophilia A].
A 74-year-old man developed a severe bleeding disorder on the basis of acquired Factor VIII (F VIII) inhibitor. Coagulation assays showed a prolonged activated partial thromboplastin time (APTT) with a normal prothrombin time (PT);F VIII level was 0.07 IU and F VIII inhibitor level 8.8 Bethesda units (BU). At least half the cases of acquired haemophilia A are associated with pregnancy, the postpartum period or an underlying malignancy or autoimmune disease. Haemorrhagic diathesis can be severe and life-threatening. Treatment of acute haemorrhages consists of human or porcine factor VIII concentrate, activated prothrombin complex concentrate (FEIBA) or recombinant factor VIIa, depending on the antibody titre. Immunosuppressive therapy is successful in at least 60% of the patients in making the inhibitor disappear. In patients with a spontaneous haemorrhagic diathesis, a thorough medical history should be taken, coagulation assays should be performed and a specialist should be consulted.
Brouwer
R E
RE
Afd. Algemene Interne Geneeskunde, Academisch Ziekenhuis, Leiden.
Huisman
M V
MV
Feuth
J D
JD
van der Meer
F J
FJ
D000368:Aged; D005169:Factor VIII / Q000037:antagonists & inhibitors*; D006467:Hemophilia A / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D008297:Male; D010314:Partial Thromboplastin Time
D005169:Factor VIII