Subarachnoid Hemorrhage in Congenital Factor X Deficiency: A Case Study and Literature Review
نویسندگان
1 Central Medical Laboratory, Ayatollah Taleghani Hospital, Tehran, Iran
2 Emergency Medicine Specialist, Department of Emergency Medicine, Ayatollah Taleghani Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
3 Department of Chemical Engineering, Biotechnology Faculty of Engineering, Payame Noor University, Tehran, Iran
4 Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
5 Hematology and Oncology Research Center, Tabriz University of Medical Sciences, Tabriz, Iran
6 Faculty of Biological Science, Islamic Azad University, North-Tehran Branch, Tehran, Iran
7 Mehr General Laboratories, Tehran, Iran
8 Department of Hematology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
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چکیده
Inborn factor X deficiency (FXD) is a very rare (1: 500,000) hereditary coagulation disorder, which is characterized by clinical manifestationsincluding hematoma, epistaxis, menorrhagia, ecchymosis, and central nervous system (CNS) or gastrointestinal (GI) bleeding (dependingon 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 theirfrequent 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) FXdeficiency including a 3-year-old boy and a 1-month-old female neonate. The world literature was explored through the PubMed Medline and Scopususing appropriate and pertinent key words.Results: The Patients referred to the hematology department due to the neurological complications such as vomiting, unconsciousness, prolongednasal bleeding for recent 12 hours. They had no familial history of spontaneous CNS bleeding. The blood coagulation test analysis indicated a prolongedactivated partial thromboplastin time (APTT) and also revealed a prolonged prothrombin time (PT) and the low levels of coagulation factorX implicating severe congenital FX deficiency. They followed up by our hematologists to prevent intracranial hemorrhage.Discussions: AsoneICHpatientwhosePTandaPTT suggest a coagulation disorder secondary to vitaminKdeficiency or coagulation factor deficiency,unresponsiveness to vitamin K therapy should be useful to take FX deficiency into consideration.
