Ann Hematol. 2025 Sep 29. doi: 10.1007/s00277-025-06625-2. Online ahead of print.
ABSTRACT
Sickle cell anemia (SCA) remains a significant cause of morbidity and mortality in sub-Saharan Africa. In The Gambia, limited data exist on the clinical severity and predictors of disease burden in affected children. This study aimed to assess severity patterns and identify factors associated with disease severity among children with SCA using a validated clinical scoring system. A hospital-based cross-sectional study was conducted at the Pediatric Hematology Clinic of Edward Francis Small Teaching Hospital, Banjul. One hundred sixty-four children aged 1-18 years with confirmed HbSS were enrolled. Clinical and demographic data were collected using a structured proforma, and disease severity was assessed using the Adegoke and Kuti scoring system. Data were analyzed with SPSS version 20. Associations between severity and clinical variables were explored using chi-square tests, t-tests, and correlation analysis. Among the 164 participants, 67.1% had mild disease, 31.7% moderate, and 1.2% severe. The mean age was 8.43 ± 4.19 years, and the male-to-female ratio was 1.34:1. Gender was significantly associated with disease severity (P = 0.028). Early age at diagnosis showed a non-significant trend toward higher severity. Painful crises, hospitalizations, and blood transfusions were significantly associated with greater severity (P < 0.001). Laboratory markers such as low packed cell volume and elevated white blood cell counts also correlated with higher severity. Acute chest syndrome was the most frequent complication (34%). Children on hydroxyurea tended to have higher severity scores, reflecting that the medication was typically initiated in those with more severe disease. Most Gambian children with SCA in this cohort exhibited mild to moderate disease. Clinical severity was significantly associated with gender, frequency of complications, and select hematologic parameters. The findings underscore the need for early diagnosis through neonatal screening and improved access to hydroxyurea and comprehensive care. Further research into genetic modifiers may enhance individualized disease management in this setting.
PMID:41016981 | DOI:10.1007/s00277-025-06625-2
