Intracranial haemorrhage in acquired haemophilia with inhibitors

Background: Acquired hemophilia with inhibitors is a serious complication in patients with autoimmune disorders. Bleeding complications usually occur in the skin or muscles, but uncommonly as intracranial hemorrhage.

Case report: A 68-year-old female was admitted to our hospital after acute onset of severe headache and drowsiness. Past diagnoses included rheumatoid arthritis in 1986 and hyperthyroidism in 2002 when she was receiving radio-iodine therapy. On admission, the patient was somnolent and showed left-sided severe sensorimotor hemiparesis, homonymous hemianopia and hemineglect to the left. In addition, we found petechiae and hematomata all over the body. The initial cranial computed tomography showed a large intracerebral hemorrhage of 8×3.3cm in the right temporal lobe. Blood tests were normal except for a significantly prolonged APTT. Because there was no drug-related reason for this finding we performed additional tests and found that factor VIII measured by an one-stage clotting assay was extremely low. The Bethesda assay was positive.

We initiated treatment of this acquired hemophilia with recombinant factor VIIa (NovoSeven™) about two hours after admission. On the same day the patient was transferred to the Neurosurgery department where open hematoma evacuation was performed over the next hours. The patient received a total of 480 kIU recombinant factor VIIa on the first day. Treatment was continued with a dosage of 60 kIU every 6 hours for the following five days.

Following surgery the patient received 250mg methylprednisolone per day for four days with a consecutive slight temporary elevation of factor VIII to 0.16 IU/ml.

On day five, we administered a single dose of 100mg cyclophosphamide and 75mg of prednisolone daily thereafter. On day eight the patient developed a stuporous state. The CCT showed a new bleeding in the right temporal lobe. Due to rapid brainstem compression another surgical intervention was not a viable option. We stopped treatment with rVIIa.

On day 17 the patient died of brainstem compression.

Discussion: Early treatment of bleeding complications is critical in patients with acquired hemophilia. This specific disorder must be taken into consideration when patients with underlying autoimmune disease present with acute intracerebral bleeding. Initial treatment with rVIIa to arrest bleeding seems to be adequate. Therapy for long-term recovery and eradication or suppression of the inhibitors is not yet sufficiently clarified.