Date Published:

Abstract:

Introduction : Among genetic diseases in hematology, β Thalassemia ranks second after sickle cell disorders in Algeria. Given the seriousness and cost of the care that arises, a national action plan is needed. As a starting point, a national survey is essential to know the epidemiological situation of this disease. Objective : To determine the soci-demographic, diagnosis and evolution characteristics of major and intermediate thalassemia in Algeria. Materiels and Methods : This is a multicenter, retrospective, analytical study of 775 patients with thalassemia major (TM: 598 cases) and intermediate (TI: 177 cases). This national survey is representative of 21 services (Hematology: 19, Pediatrics: 03). The data was collected from medical records on a survey card distributed to all relevant services. Results : As of January 1, 2017, the prevalence of β Thalassemia estimated from this study is of the order of 3.47 cases per 100 000 inhabitants. The current average age of major thalassemia (MT) is 17.90 years, with extremes of [1-44 years], that of intermediate form (IT) is 23 years old with extremes of [1-61 years], the sex ratio is 1.15. The concept of consanguinity is specified in 499 patients and is present in 49.30% of cases, the geographical origin of patients is variable, 53% are from the center of the country and 43% from the east. The circumstances of the diagnosis are known in 87.25% of subjects, these are clinical symptoms in 93.91% of patients, with an average age at diagnosis of 16.31 months for MT and 5.5 years for IT. The diagnosis is neonatal in only 5.68% of cases. Blood cells transfusion needs are known in 80% of our cohort, 86% receive a systematic and regular diet; 554 patients are on chelation therapy, the main modality being deferoxamine or Deferasirox type monotherapy. 40 MT patients received Hematopoetic Stem Cell Transplantation. The monitoring of iron overload was based mainly on the determination of ferritinemia, only 8.8% of patients received cardiac and hepatic MRI. Various complications associated with iron overload have been reported, the most common being: Hepatic injury (48%), heart disease (36%), diabetes (30%), hypothyroidism (29.17). %), with average ages of onset of 26.67, 18, 19, and 14.50 years, respectively. In addition, viral serology was performed in 536 patients, 64 (11.9%) of whom were carriers of anti-HCV Ab. Conclusion : The quality and life expectancy of thalassemic patients has improved significantly in recent years; complications related to iron overload remain the leading cause of death, the means of evaluation remain insufficient in our country. In addition, the care of our patients must be standardized on the national territory. Disclosures No relevant conflicts of interest to declare.