TITLE:
Characterization of Bleeding and Laboratory Phenotype in Hemophilia A Carriers: A Cross-Sectional Study in Benin
AUTHORS:
Tatiana Baglo Agbodandé, Alban Zohoun, Abdel Aziz Ouabi, Edwige Araba, Bienvenu Houssou, Romaric Massi, Charlotte Orou Guiwa, Ludovic Anani, Dorothée Kindé Gazard, Awa Oumar Touré Fall
KEYWORDS:
Carrriers of Hemophilia A, Bleeding Symptoms, APTT, Factor VIII, FVIII:C/FvW:Ag ratio
JOURNAL NAME:
Open Journal of Blood Diseases,
Vol.13 No.4,
December
14,
2023
ABSTRACT: Background: In Africa, hemophilia is underdiagnosed and carriers have long been
considered free from bleeding symptoms. However, recent research has begun to
reveal hemostatic abnormalities and bleeding manifestations in carriers of
hemophilia A, particularly due to excessive inactivation of normal X
chromosomes. Objective: To describe the bleeding symptoms and hemostatic
abnormalities in carriers of hemophilia A (HA) in Benin. Methods: This
study was conducted as a prospective cross-sectional investigation between April 2021 to March 2022. The study population
consisted of identified through pedigrees of persons with hemophilia A being treated in various
hospitals in Benin. Data
were collected through interviews conducted by trained physician and each carrier underwent
a biological workup. Results: A total of 71 hemophilia A carriers were
included and 38 of whom were obligatory carriers. Thirty-one carriers (43.7%)
reported abnormal bleeding symptoms. Menorrhagia
has (71%) being the most important manifestation, followed by bleeding
during or after childbirth (45.2%). Among the 71 carriers, 45 were of
reproductive age. Of whom 22 (48.8%) had a Higham score exceeding 100. Activated
partial thromboplastin time was prolonged in 7 carriers (9.9%). The mean
activity factor VIII:C (FVIII:C) levels were 68.8 ± 34.9
IU/dL. The
average FVIII:C level in obligatory carriers was 56.9% and among potential carriers, the average FVIII:C level was higher at
80.4%. However twelve female carriers (16.9%) had FVIII:C levels :C/FvWAg ratio was below 0.7 in 73.2% of female
drivers. Obligatory carriers (p = 0.00003) and FVIII; C/FvWAg
ratio = 0.003) were statistically associated with abnormal
bleeding symptoms, while blood group O (p = 0.0002) and FVIII/FvWAg ratio = 0.0016) were associated
with a higher risk of menorrhagia. Conclusion: In Benin, carriers of
haemophilia A present bleeding symptoms and haemostatic abnormalities. Further
studies on a larger number of carriers are needed to better characterize and
manage these patients.