Intrakranielle Blutung bei erworbener Hemmkörperhämophilie

Abstract

Acquired hemophilia is a rare complication in autoimmune disorders and malignancies. It can result in bleedings into skin and muscle, whereas intracranial hemorrhage in adults has so far not been described. We report a patient with acute intracerebral hemorrhage due to acquired hemophilia with factor VIII inhibition. The patient was treated with recombinant factor VIIa and open hematoma evacuation followed by administration of cortisone and cyclophosphamide. After good initial recovery, intracerebral rebleeding occurred and the patient died from brainstem compression.

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

“…In 2003 and 2006, 2 AH patients were reported who first presented intracranial haemorrhage. One of them showed good recovery with rFVIIa whereas the other died due to intracerebral rebleeding, despite having received FVIIa treatment [11,12].…”

Acquired Hemophilia First Manifesting as Life-Threatening Intracranial Hemorrhage -Case Report-

“…Of the 2 previously reported patients who first presented with intracranial hemorrhage, one showed good recovery with rFVII, prednisolone, and cyclophosphamide treatment, 1) but the other died of rebleeding after transient recovery with the same treatment. 7) Further investigation is needed to optimize the treatment options for acquired hemophilia in patients presenting with intracranial hemorrhage.…”

“…8) Only two cases of intracranial hemorrhage with acquired hemophilia have been reported. 1,7) Here we report a case of severe intracranial hemorrhage associated with preoperative prolongation of activated partial thromboplastin time (APTT) caused by the presence of autoantibodies to FVIII.…”