An epileptic seizure and haemorrhage into the ventricular system of the brain as the first manifestations of acquired haemophilia A - Case report

Ann Agric Environ Med. 2021 Sep 16;28(3):531-533. doi: 10.26444/aaem/140306. Epub 2021 Sep 10.

Abstract

Acquired haemophilia (AH) is a suddenly occurring severe blood diathesis that affects both males and females and is caused by autoantibodies which inhibit coagulation factor VIII. The report describes an unusual case of acquired haemophilia in which an epileptic seizure and haemorrhage into the ventricular system of the brain were the first manifestations of the disease. In addition, APTT was prolonged to 94.6 seconds and the factor VIII level was as low as 1.5%. The level of anti-FVIII antibody was extremely high - 272BU/ml. The patient did not undergo invasive diagnostic procedure or an operation. Recombinant factor VIIa was used to control the bleeding. In order to eradicate the inhibitor, the patient received prednisone and cyclophosphamide. Complete remission was achieved after 5.5 weeks of treatment.

Keywords: factor VIII; hemophilia; intracranial hemorrhages.

Publication types

  • Case Reports

MeSH terms

  • Autoantibodies / blood
  • Cerebral Ventricles / blood supply*
  • Cerebral Ventricles / diagnostic imaging
  • Factor VIII / metabolism
  • Hemophilia A / complications*
  • Hemophilia A / diagnostic imaging
  • Hemophilia A / metabolism
  • Hemophilia A / pathology
  • Hemorrhage / diagnostic imaging
  • Hemorrhage / etiology
  • Hemorrhage / pathology
  • Humans
  • Male
  • Middle Aged
  • Seizures / blood
  • Seizures / etiology*
  • Seizures / pathology

Substances

  • Autoantibodies
  • F8 protein, human
  • Factor VIII