| dc.description.abstract | Background: Inborn factor X deficiency (FXD) is a very rare (1: 500,000) hereditary coagulation disorder, which is characterized by clinical manifestations including hematoma, epistaxis, menorrhagia, ecchymosis, and central nervous system (CNS) or gastrointestinal (GI) bleeding (depending on the zygosity). In homozygote patients, the risk of spontaneous intracranial hemorrhage (ICH) is high. Objectives: The aim of this investigation was to study and long-term follow-up of the patients with FXD and ICH. In addition, we investigated their frequent bleeding symptoms throughout their life and the results were compared with results of other studies. Patients and Methods: This study investigated 2 cases with spontaneous intracranial hemorrhage in patients with severe congenital (factor X) FX deficiency including a 3-year-old boy and a 1-month-old female neonate. The world literature was explored through the PubMed Medline and Scopus using appropriate and pertinent key words. Results: The Patients referred to the hematology department due to the neurological complications such as vomiting, unconsciousness, prolonged nasal bleeding for recent 12 hours. They had no familial history of spontaneous CNS bleeding. The blood coagulation test analysis indicated a prolonged activated partial thromboplastin time (APTT) and also revealed a prolonged prothrombin time (PT) and the low levels of coagulation factor X implicating severe congenital FX deficiency. They followed up by our hematologists to prevent intracranial hemorrhage. Discussions: As one ICH patient whose PT and a PTT suggest a coagulation disorder secondary to vitamin K deficiency or coagulation factor deficiency, unresponsiveness to vitamin K therapy should be useful to take FX deficiency into consideration. | |
