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

Rationale: Coagulation factor deficiencies can lead to hemorrhagic syndromes of varying severity and bleeding manifestations. Intracranial hemorrhage (ICH) associated with coagulation factor XIII (FXIII) deficiency is particularly challenging because its clinical presentation closely mimics spontaneous ICH, often occurring without apparent triggers. Routine coagulation tests (prothrombin time and activated partial thromboplastin time) are typically normal, contributing to diagnostic difficulties and frequent underdiagnosis or misclassification. As the management of this condition differs from other ICH types, replacement therapy with cryoprecipitate or fresh frozen plasma is crucial beyond surgical intervention. This case report highlights this rare, life-threatening condition to enhance clinical recognition. Patient concerns: A 53-year-old Chinese male presented with recurrent ICH over a 20-day period and underwent 2 intracerebral hematoma evacuation surgeries. Postoperative head computed tomography (CT) revealed persistent rebleeding at the site of original hemorrhage. Diagnoses: Congenital FXIII deficiency, intracerebral hemorrhage, and cerebral herniation. Interventions: The patient underwent surgical hematoma evacuation followed by allogeneic blood transfusion therapy, and received 10 units of cryoprecipitate daily. Outcomes: 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. He was discharged in stable condition. Post-discharge management included weekly infusions of 6 to 10 units of cryoprecipitate at local medical facilities along with routine monitoring of coagulation profiles and cranial CT scans. Lessons: Intracranial hemorrhage secondary to FXIII deficiency is exceedingly rare. Clinicians should consider FXIII deficiency when recurrent ICH occurs with normal routine coagulation tests, to enable early diagnosis and life-saving replacement therapy. Early identification of the underlying etiology through comprehensive diagnostic evaluation is essential to guide effective and timely interventions, particularly in the context of increasing ICH incidence and healthcare resource utilization.