PB0710 - Clinical Manifestations and Bleeding Scores in Inherited Factor VII Deficiency; The first national report from Iran.

Monday, July 11, 2022

6:30 PM – 7:30 PM

Location: Exhibition

Presentation Format: In-Person

Co-Author(s)

Abbas Khosravi, PhD

Ph.D Student
*Transfusion Research center, High Institute for Research and Education in Transfusion Medicine, Tehran ,Iran
Tehran, Tehran, Iran
MP

Mostafa Paridar

Academic Member
Ministry of health and medical edjucation,Tehran,Iran
Tehran, Tehran, Iran
MK

Mehraneh Karimzadeh

Resident
Department of Dermatology, Rasool Akram Medical Complex Clinical Research Development Center (RCRDC), School of Medicine, Iran University of Medical Sciences, Tehran, Iran
Tehran, Tehran, Iran
VT

Vahideh Takhviji

PH.D Student
Transfusion Research center, High Institute for Research and Education in Transfusion, Tehran, Iran.
Tehran, Tehran, Iran
AK

Ali Kordian

Dentist
Ahvaz University of Medical Sciences, Ahvaz, Iran
Tehran, Tehran, Iran
OK

Omid Kiani Ghalehsardi

Ph.D Student
Iran University of Medical Sciences, Tehran, Iran
Tehran, Tehran, Iran

Presenting Author(s)

AK

Anahita Kalantari

Dr
Department of Anesthesiology, Ahvaz Jundishapur University of Medical Sciences
Ahvaz, Khuzestan, Iran

Background: Factor VII deficiency is the most frequent disorder among “rare inherited bleeding disorders” and is considered as a heterogeneous disorder, on the phenotype and genotype levels. The majority of studies have been focused on the genetic characteristics.

Aims: The present study investigates the bleeding patterns and their severity attitude regarding multiple clinical, laboratory, and demographic properties in Factor VII (FVII) deficient patients.

Methods: Demographic characteristics, clinical manifestations, family history, and treatment history were recorded, along with specific tests. By recording 12 bleeding symptoms in the year prior to inclusion, the severity of those symptoms was assessed using ISTH Bleeding Scoring.

Results: Data revealed a varied pattern in bleeding symptoms (mild to life-threatening) and FVII level in sex, age of manifestation, and the level of FVII (severe: 7.4%, moderate: 35%, mild: 52.6%). Bleeding tendency was significantly associated with FVII deficiencies. In fact, the odds of bleeding doubled with decreasing FVII levels, even after adjusting for other factors. Moreover, there were significant correlations between all the symptoms and their severities and sex and age subgroups. Life-threatening symptoms (CNS and GI) were more prevalent in pediatrics than adults that showed significantly higher cutaneous bleeding types. The incidence of all symptoms was significantly higher in women especially mucocutaneus bleedings.

Conclusion(s): The assessment of bleeding tendency and severity in FVII deficiency should not be limited to ISTH scores, but rather based on clinical manifestations, laboratory results, and demographic factors.