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

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