Author/Authors :
Mohammadi، Sepideh نويسنده Department of Microbiology, Islamic Azad University of Pharmaceutical Sciences Branch, Tehran, Iran , , Torab، Zahra نويسنده Hematology and Oncology Research Center, Tabriz University
of Medical Sciences, Tabriz, Iran , , Aghakhani، Soheila نويسنده Faculty of Biological Science, Islamic Azad University,
North-Tehran Branch, Tehran, Iran , , Ghalandari، Mina نويسنده Emergency Medicine Specialist, Department of Emergency
Medicine, Ayatollah Taleghani Hospital, Shahid Beheshti University
of Medical Sciences, Tehran, Iran , , Mohammadimanesh، Reyhaneh نويسنده Department of Chemical Engineering, Biotechnology Faculty
of Engineering, Payame Noor University, Tehran, Iran , , Asgary، Vahid نويسنده Department of Immunology, School of medicine, Tehran University of Medical Sciences, Tehran, Iran Asgary, Vahid , Louni Aligoudarzi، Samira نويسنده Mehr General Laboratories, Tehran, Iran , , Younesi، Mohammad Reza نويسنده MSc of Hematology ,
Abstract :
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. 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. 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. 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. As
one ICH patient whose PT and aPTT 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.
