Recurrent cortical intracranial hemorrhage secondary to factor XIII deficiency: case report and literature review

Factor XIII (FXIII) deficiency is a rare coagulation disorder that can present in a variety of clinical pictures, including umbilical cord bleeding, poorly healed wounds, and intracranial hemorrhage (ICH). Most of the patients also have prolonged bleeding issues. Here, we present a case of recurrent ICH from the same site due to FXIII deficiency.

The patient is a 24-year-old man who presented with a thunderclap headache and homonymous hemianopia to the hospital. His medical history was only significant for a prior ICH a decade ago. Blood workups were within normal limits. Magnetic resonance imaging (MRI) revealed a heterogeneous, high- to iso-signal in T2 and iso- to high-signal in T1 lesion in the occipital lobe. An angiography was performed, revealing no vascular lesions or pathologic findings. 4 days after the angiography, the hematoma expanded, and the patient started bleeding from the catheter insertion site. After a hematology consultation and running specific tests, FXIII deficiency was detected. Cryoprecipitate therapy for the patient was started, and he was advised to undergo routine FXIII level assessment.

This case shows the importance of considering coagulation disorders, including FXIII deficiency, in recurrent ICHs, especially when other findings are normal.