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Characterization of Bleeding and Laboratory Phenotype in Hemophilia A Carriers: A Cross-Sectional Study in Benin

Open Journal of Blood Diseases 2023年第4期13卷 148-159页

作者： 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 Hematology Laboratory National Teaching Hospital Hubert KoutoukouMaga of Cotonou Cotonou Benin Faculty of Health Sciences University of Abomey-Calavi Cotonou Benin Hematology Laboratory of The Aristide le Dantec Hospital Dakar Senegal Pharmacy and Odontostomatology Faculty of Medicine Cheikh Anta Diop University of Dakar Dakar Senegal

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 < 40%. The FVIII: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 carrie

关键词： Carrriers of Hemophilia A Bleeding Symptoms APTT Factor VIII FVIII:C/FvW:Ag ratio

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Prevalence of Association of Glucose-6-Phosphate Dehydrogenase Deficiency and Sickle Cell Disease at the National Teaching Hospital of Cotonou in Benin

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Open Journal of Blood Diseases 2023年第3期13卷 93-101页

作者： Alban Zohoun Tatiana Baglo-Agbodande Thierry Adje Bienvenu Houssou Romaric Massi Gnon Gourou orou guiwa Justin Dèhoumon Josiane Mehou Ludovic Anani Anne Vovor Dorothée Kinde-Gazard Laboratoire d’Hématologie Clinique Universitaire des Maladies du Sang CNHU-HKM Cotonou Benin Faculté des Sciences de la Santé de Cotonou Université d’Abomey Calavi Cotonou Benin Faculté des Sciences de la Santé Université de Lomé Lomé Togo

Background: Glucose-6-phosphate dehydrogenase (G6PD) deficiency and sickle cell disease are common genetic defects of red blood cells that lead to hemolytic anemia. The prevalence of G6PD deficiency in sickle cell patients is unknown in Benin. Objective: This study aimed to determine the prevalence of G6PD deficiency in sickle cell patients at the CNHU-HKM of Cotonou. Methods: This prospective study was conducted from April to November 2022 at the blood-related diseases teaching clinic and included sickle cell patients in the stationary phase. G6PD determination was performed using the enzymatic method on a Mindray BS 200 machine following the Herz method. Hematological parameters were determined using the XT 4000i analyzer and supplemented by a blood smear stained with May Grunwald Giemsa. Data were analyzed using Epi Info 3.5.4 software. Results: One hundred and sixty-four sickle cell patients (80 SS homozygotes and 84 SC heterozygotes) in the intercritical phase, with a mean age of 26.30 ± 10.76 years, were included. The prevalence of G6PD deficiency was 9.1% (15 cases found in 7 SS patients and 8 SC patients). In G6PD-deficient patients, the mean concentration of the enzyme was lower in Hb SC heterozygotes than in Hb SS homozygotes: 3.56 IU/g Hb versus 4.98 IU/g Hb. The mean reticulocyte count was 231.43 G/L in the deficient group, compared to 216.32 G/L in the non-deficient group. Conclusion: The preliminary results of our study reveal a high prevalence of G6PD deficiency in sickle cell patients. The impact of this association on hematologic and biological parameters should be evaluated for better management of sickle cell disease.

关键词： Sickle Cell Disease G6PD Deficiency Prevalence Hemogram

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