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

1*Transfusion Research center, High Institute for Research and Education in Transfusion Medicine, Tehran ,Iran, Tehran, Tehran, Iran, 2Ministry of health and medical edjucation,Tehran,Iran, Tehran, Tehran, Iran, 3Shahid Beheshti University of Medical Sciences, Tehran, Iran, Tehran, Tehran, Iran, 4Transfusion Research center, High Institute for Research and Education in Transfusion Medicine, Tehran ,Iran, Tehran, Tehran, Iran, 5Ahvaz University of Medical Sciences, Ahvaz, Iran, Tehran, Tehran, Iran, 6Iran University of Medical Sciences, Tehran, Iran, Tehran, Tehran, Iran, 7Department of Anesthesiology, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Khuzestan, Iran

Abstract Number: PB0710

Meeting: ISTH 2022 Congress

Theme: Hemophilia and Rare Bleeding Disorders » Rare Bleeding Disorders

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.

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Pattern of bleeding symptoms in children compared to adult

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Comparison of bleeding symptoms among severe, moderate and mild groups based on the decrease in plasma level of FVII in the year preceding the inclusion in the study.

To cite this abstract in AMA style:

Khosravi A, Paridar M, Karimzadeh M, Takhviji V, Kordian A, Kiani Ghalehsardi O, Kalantari A. Clinical Manifestations and Bleeding Scores in Inherited Factor VII Deficiency; The first national report from Iran. [abstract]. https://abstracts.isth.org/abstract/clinical-manifestations-and-bleeding-scores-in-inherited-factor-vii-deficiency-the-first-national-report-from-iran/. Accessed September 29, 2023.

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ISTH Congress Abstracts - https://abstracts.isth.org/abstract/clinical-manifestations-and-bleeding-scores-in-inherited-factor-vii-deficiency-the-first-national-report-from-iran/