Recurrent Spontaneous Intracerebral Haemorrhage Due To Factor XIII Deficiency

While hypertension is the most common cause of primer intracerebral hemorrhage, various rare causes can be cited including hematologic diseases. In this report, a case with recurrent intracerebral hemorrhage due to a rare coagulation defect, factor XIII deficiency is presented. Thirty six year old female patient was admitted to the neurology clinic with sudden onset nausea, vomiting, unconsciousness and right hemiplegia. A large hematoma was seen at the left frontoparietal region on computed brain tomography (CT). CT angiography was normal. All the laboratory tests were normal, except factor XIII clot solubility test being positive. Antiedema and fresh frozen plasma therapy (FFP) were given for several days. After stabilization of the clinical picture, the patient was discharged with motor aphasia and slight right hemiparesis. One month later the patient was seen with normal neurological examination. However, three months later she was admitted to the hospital again with severe headache. CT was repeated and in the parietal lobe, a new hematoma was seen. She was treated with FFP in the same way and discharged 2 weeks later with nominal aphasia. In spontaneous and recurrent ICH cases, hematologic causes must be kept in mind albeit very rare, especially in the young. It is important to identify coagulation disorders among hematologic causes as their therapy is possible and life saving.