PAEDIATRIC SICKLE CELL DISEASE: HISTOPATHOLOGICAL IMPACT ON ORGAN SYSTEMS AND PHYSIOLOGICAL MARKERS OF DISEASE SEVERITY AND ITS TREATMENT.

To evaluate histopathological organ involvement and associated physiological markers in children with SCD, and to assess treatment patterns and clinical response. Methods A cross-sectional study was conducted at the Burns and Plastic Surgery Centre, Hayatabad, from January 2023 to January 2024, including 73 children with confirmed SCD. Demographic, clinical, histopathological, and laboratory data were recorded. Statistical analysis was performed using SPSS version 26, with p<0.05 considered significant. Results The 5–10 years age group was the most affected (43.8%), with a male predominance (56.2%). Splenic fibrosis/autosplenectomy (57.5%), hepatic congestion with hemosiderosis (52.1%), and bone marrow erythroid hyperplasia (74.0%) were common histopathological findings. Laboratory results showed low hemoglobin (7.9 ± 1.2 g/dL), high reticulocyte counts (6.2 ± 1.5%), elevated LDH (680 ± 105 U/L), and hyperbilirubinemia (3.1 ± 0.9 mg/dL). Hydroxyurea was prescribed to 71.2% of patients, with a good clinical response in 67.1%. Conclusion Paediatric SCD in our cohort was characterized by early and significant multi-organ pathology, pronounced hemolytic markers, and favorable outcomes in patients receiving hydroxyurea. These findings highlight the importance of early diagnosis, comprehensive monitoring, and access to disease-modifying therapy.