Abstract
Background:Various national studies and some multicenter studies have been conducted in the past, highlighting the different foci of hemoglobinopathies. The North‐East of the country, essentially the border area with Tunisia, is an endemic area of sickle cell disease, posing many public health problems.Aims:The objectives of our work are: Census of hemoglobinopathies supported at our level Determine their epidemiological and evolutionary characteristics Methods:This is a retrospective study, extending from January 1988 to December 2017. A regular monitoring is carried out at varying rates depending on the type of hemoglobinopathy with annual impact assessments.Results:during this period a total of 1709 cases of hemoglobinopathies, was followed and each type its epidemiological characteristics: Major sickle cell syndromes: 1550 cases (90.7%) Homozygous form: 960 cases (61.94%) Double heterozygosity S / C: 99 cases (6.4%) Double heterozygosity S / B Thalassemia: 491 cases (31.68%) Epidemiological data: the current average age: 30.3 years [1 ‐ 69 years], the Sex ratio: 0.84, the majority of our patients come from Algerian‐Tunisian border area (79%). Inbreeding is present in 35.35% of cases, 70% of patients had at least one similar case in siblings. Hemoglobin C: 70 cases (4.1%) Epidemiological data: the current average age: 36 years [11 ‐ 69 years]; the Sex ratio: 0.70. Inbreeding is present in 59.69% of cases, 63% of patients had at least one similar case in siblings. The β Thalasemia: 89 cases (5.2%) Major form: 68 cases (76.4%) Intermediate form: 21 cases (23.6%) The epidemiological characteristics: the current average age: 19 years [03 ‐31 years], Sex ratio: 1.04. Inbreeding is present in 51% of cases and is 1st degree in 33.33%.Summary/Conclusion:Hemoglobin abnormalities are common in our region, mainly major sickle cell syndromes, posing a major health problem. Many efforts must be made at different levels to reduce the frequency of these abnormalities in this region and to provide adequate and above all multidisciplinary care for these patients.
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