Bioclinical features of haemophilia patients in Benin in 2023: Towards better care.

Autor: Baglo T; Haematology laboratory, National Teaching Hospital Hubert Koutoukou Maga, Cotonou, Benin.; Faculty of Health Sciences, University of Abomey-Calavi, Abomey-Calavi, Benin., Zohoun A; Haematology laboratory, National Teaching Hospital Hubert Koutoukou Maga, Cotonou, Benin.; Faculty of Health Sciences, University of Abomey-Calavi, Abomey-Calavi, Benin., Mohamed FA; Faculty of Medicine, Mother and Child Department, University of Parakou, Parakou, Benin., Araba F; Haematology laboratory, National Teaching Hospital Hubert Koutoukou Maga, Cotonou, Benin., Houssou B; Haematology laboratory, National Teaching Hospital Hubert Koutoukou Maga, Cotonou, Benin., Anani L; Haematology laboratory, National Teaching Hospital Hubert Koutoukou Maga, Cotonou, Benin.; Faculty of Health Sciences, University of Abomey-Calavi, Abomey-Calavi, Benin., Kindé-Gazard D; Haematology laboratory, National Teaching Hospital Hubert Koutoukou Maga, Cotonou, Benin.; Faculty of Health Sciences, University of Abomey-Calavi, Abomey-Calavi, Benin., Fall AT; Haematology laboratory, Aritide le Dantec Hospital, Dakar, Senegal.; Pharmacy and Odontostomatology, Faculty of Medicine, Cheikh Anta Diop University of Dakar, Dakar, Senegal., Ryman A; Laboratory of Hematology, Bordeaux University Hospital, Pessac, France., Gruel Y; University of Tours, Inserm U1327, ISCHEMIA, Tours, France., Pouplard C; University of Tours, Inserm U1327, ISCHEMIA, Tours, France.; Department of Haemostasis, University Hospital of Tours, Tours, France.
Jazyk: angličtina
Zdroj: Haemophilia : the official journal of the World Federation of Hemophilia [Haemophilia] 2024 Sep; Vol. 30 (5), pp. 1210-1216. Date of Electronic Publication: 2024 Aug 08.
DOI: 10.1111/hae.15082
Abstrakt: Objective: To analyse the demographic, clinical and laboratory data of Beninese patients with haemophilia.
Method: A prospective survey was conducted in three different hospitals of Benin from April 2021 to March 2022, to analyse clinical and biological features of patients with haemophilia previously diagnosed or identified based on personal/family history.
Results: A total of 101 patients were studied, 97 with haemophilia A and 4 with haemophilia B, including 26 new cases identified after family investigation. Their median age was 11 years, and the most frequent initial manifestations were cutaneous-mucosal haemorrhages (29.70%) and post-circumcision haemorrhages (25.74%). Previous joint bleedings were present in 77% of them, with an arthropathy in 65 cases, which particularly affected the knees (75%), elbows (41%) and ankles (29%). Factor VIII (FVIII) levels combined with activated partial thromboplastin time (APTT) values did not always enable, as would be expected, the distinction between severe and moderate haemophilia, since they were >1 IU/dl in 31 of 74 patients with APTT > 80 s, and between 1 and 2 IU/dl in 26 other cases with previous joint haemorrhages, including 18 with chronic arthropathy. Therefore, for these patients, severe haemophilia could not be excluded, and this uncertainty probably reflects technical difficulties affecting the pre-analytical and analytical stages of the APTT and FVIII/IX assays.
Conclusion: Our study proved that haemophilia is a significant reality in Benin, but also remains under-diagnosed in some districts of the country. In addition, more reliable biological tests are needed in the future to better define the severity of the disease and improve treatment of patients.
(© 2024 John Wiley & Sons Ltd.)
Databáze: MEDLINE
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