Sokołowska et al reported the rare case of a 57-year-old man diagnosed with acquired hemophilia (AH) whose first clinical manifestations were epileptic seizure and hemorrhage into the ventricular system of the brain. This unusual case was recently published in the Annals of Agricultural and Environmental Medicine.

The patient had a history of cerebral infarction, alcoholism, and scabies. At the time of admission, the man presented with an altered state of consciousness suggestive of alcohol consumption. Serum analysis confirmed an increased concentration of ethyl acid (2.22‰, physiologic value <0.2‰). There was no sign of focal impairment of the central nervous system on neurological examination.

Moreover, the patient presented with edema, subcutaneous hematomas of the left upper limb, and widespread eczema.


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During the first day of hospitalization, the man experienced an epileptic seizure, which was treated with diazepam. Brain computed tomography (CT) scans showed intraventricular hemorrhage around the septum pellucidum.

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During the second day of hospitalization, the patient developed subcutaneous hematomas of the right upper limb. The authors stated, “In the laboratory investigations, [activated partial thromboplastin time (APTT)] was so significantly prolonged that it was not possible to determine the APTT value (out of limit). The consecutive evaluation revealed the APTT value of 94.6 sec.”

The diagnosis of AH was confirmed after laboratory testing. The factor VIII (FVIII) levels were decreased (1.5%), while the anti-FVIII antibody levels were increased (272 Bethesda units/mL). The patient was treated with recombinant factor VIIa.

The authors concluded, “The situation presented above teaches us that in patients with no medical history of bleeding a sudden [occurrence] of intracranial hemorrhage should arouse the suspicion of AH, especially if isolated prolonged APTT is also found.”

After 3 weeks of recombinant factor VIIa treatment (total dose, 227 mg), the patient received prednisone
and cyclophosphamide. Hemostatic and immunosuppressive agents were also well tolerated. The patient was considered to be in complete remission after 5.5 weeks and was discharged from the hospital with a prednisone regimen.

Reference

Sokołowska B, Kozińska J, Szczepanek D, et al. 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;28(3):531-533. doi:10.26444/aaem/140306