Recurrent intracranial hemorrhage secondary to congenital factor XIII deficiency: A case report and literature review.

A 53-year-old male with congenital factor XIII deficiency presented with recurrent intracranial hemorrhage over a 20-day period requiring two surgical evacuations.

• The patient was a 53-year-old Chinese male.
• He experienced recurrent ICH within a 20-day period.
• He underwent 2 intracerebral hematoma evacuation surgeries.
• Postoperative head CT revealed persistent rebleeding at the site of original hemorrhage.
• The patient developed cerebral herniation as a complication.

Routine coagulation tests (prothrombin time and activated partial thromboplastin time) were normal in this patient with FXIII deficiency, contributing to diagnostic difficulty.

• Routine coagulation tests including prothrombin time and activated partial thromboplastin time are typically normal in FXIII deficiency.
• Normal routine coagulation tests contribute to frequent underdiagnosis or misclassification of FXIII deficiency.
• The clinical presentation closely mimics spontaneous ICH, often occurring without apparent triggers.
• This diagnostic difficulty underscores the need to specifically test for FXIII when recurrent ICH occurs with normal routine coagulation studies.

Treatment with surgical hematoma evacuation combined with cryoprecipitate replacement therapy resulted in full neurological recovery.

• The patient received surgical hematoma evacuation followed by allogeneic blood transfusion therapy.
• He received 10 units of cryoprecipitate daily as replacement therapy.
• After 35 days of treatment, the patient regained full consciousness with normal cognitive and neurological responsiveness.
• Follow-up CT imaging revealed significant resolution of the hematoma.
• The patient was discharged in stable condition.

Post-discharge management for congenital FXIII deficiency-associated ICH included regular prophylactic cryoprecipitate infusions and monitoring.

• Post-discharge management included weekly infusions of 6 to 10 units of cryoprecipitate at local medical facilities.
• Routine monitoring of coagulation profiles and cranial CT scans was also prescribed.
• Replacement therapy with cryoprecipitate or fresh frozen plasma is described as crucial beyond surgical intervention.
• The management of FXIII deficiency-associated ICH differs from other ICH types.

Intracranial hemorrhage secondary to FXIII deficiency is described as exceedingly rare and life-threatening.

• The paper characterizes ICH secondary to FXIII deficiency as 'exceedingly rare' and 'particularly challenging.'
• The condition is noted to be life-threatening.
• The case report was written to 'highlight this rare, life-threatening condition to enhance clinical recognition.'
• The paper emphasizes the importance of 'early identification of the underlying etiology through comprehensive diagnostic evaluation' particularly in the context of increasing ICH incidence and healthcare resource utilization.