Homozygous Sickle Cell Disease after Age of 40: Follow-Up of a Cohort of 209 Patients in Senegal, West Africa.

Abstract

Objectives: The aim of this study was to describe the morbidity and mortality of homozygous sickle cell disease after the age of 40.

Methods: This was a cohort study of 209 patients followed from 1994 to 2022. All hemoglobin electrophoresis-confirmed SS sickle cell patients over 40 years were included. A descriptive study of epidemiological, diagnostic, therapeutic, and evolutionary data was used to assess morbidity and mortality.

Results: Sex ratio (M/F) was 0.6. Median age was 47 (41-75). According to morbidity, 95.1% had less than 3 vaso-occlusive crises/year. Acute anemia was the most frequent complication (52.63%). Chronic complications were noted in 32.5%. At diagnosis, mean hemoglobin was 8.1 g/dl ± 1.9, HbS was 86.5 ± 10, and HbF was 9.4 ± 7.6. Number of patients transfused was 66%. We noted that 8.1% of patients died, 29.2% were lost to follow-up, and 62.7% were still being followed up. The risk factors identified for death were geographical origin, comorbidity, high HbS, low HbF, and thrombocytosis.

Conclusion: This study shows that homozygous SCD is increasingly becoming an adult disease and that it can be carried into old age in Africa. Advanced age over 40 is marked by an upsurge in chronic complications, making it essential to set up a screening program and to organize multidisciplinary follow-up.