PAEDIATRIC SICKLE CELL DISEASE: HISTOPATHOLOGICAL IMPACT ON ORGAN SYSTEMS AND PHYSIOLOGICAL MARKERS OF DISEASE SEVERITY AND ITS TREATMENT.
Main Article Content
Keywords
Sickle cell disease, Pediatrics, Histopathology, Organ damage, Hemolysis, Hydroxyurea, Hemoglobinopathy
Abstract
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.
References
2. Vona, R., et al., Sickle cell disease: role of oxidative stress and antioxidant therapy. 2021. 10(2): p. 296.
3. Silva, M. and P.J.A. Faustino, From stress to sick (le) and back again–oxidative/antioxidant mechanisms, genetic modulation, and cerebrovascular disease in children with sickle cell anemia. 2023. 12(11): p. 1977.
4. Tebbi, C.K.J.H., Sickle cell disease, a review. 2022. 3(2): p. 341-366.
5. Vinhaes, C.L., et al., Hydroxyurea treatment is associated with reduced degree of oxidative perturbation in children and adolescents with sickle cell anemia. 2020. 10(1): p. 18982.
6. Idris, I.M., et al., Sickle cell disease as an accelerated aging syndrome. 2022. 247(4): p. 368-374.
7. Sahu, T., et al., Infection and potential challenge of childhood mortality in sickle cell disease: a comprehensive review of the literature from a global perspective. 2023. 13(3): p. 206-229.
8. Aboderin, F.I., et al., A review of the relationship between the immune response, inflammation, oxidative stress, and the pathogenesis of sickle cell anaemia. 2023. 11(9): p. 2413.
9. Darbari, D.S., V.A. Sheehan, and S.K.J.E.j.o.h. Ballas, The vaso‐occlusive pain crisis in sickle cell disease: definition, pathophysiology, and management. 2020. 105(3): p. 237-246.
10. Gbotosho, O.T., M.G. Kapetanaki, and G.J.J.F.i.i. Kato, The worst things in life are free: the role of free heme in sickle cell disease. 2021. 11: p. 561917.
11. Sachdev, V., D.R. Rosing, and S.L.J.T.i.c.m. Thein, Cardiovascular complications of sickle cell disease. 2021. 31(3): p. 187-193.
12. Ochocinski, D., et al., Life-threatening infectious complications in sickle cell disease: a concise narrative review. 2020. 8: p. 38.
13. Longoria, J.N., et al., Neurocognitive risk in sickle cell disease: Utilizing neuropsychology services to manage cognitive symptoms and functional limitations. 2022. 197(3): p. 260-270.
14. Ataga, K.I., S.L. Saraf, and V.K.J.N.R.N. Derebail, The nephropathy of sickle cell trait and sickle cell disease. 2022. 18(6): p. 361-377.
15. Akhter, M.S., et al., Mitochondria: emerging consequential in sickle cell disease. 2023. 12(3): p. 765.
16. Jacob, S.A., et al., The evolving treatment landscape for children with sickle cell disease. 2023. 7(11): p. 797-808.
17. Shet, A.S., M.A. Lizarralde-Iragorri, and R.P.J.H. Naik, The molecular basis for the prothrombotic state in sickle cell disease. 2020. 105(10): p. 2368.
18. Palomarez, A., et al., Cardiovascular consequences of sickle cell disease. 2022. 3(3).
19. Helvaci, M.R., et al., An exaggerated capillary endothelial edema may be the cause of sudden deaths in sickle cell diseases. 2023. 7(10).
20. Helvaci, M.J.W., Acute painful crises may be causes of sudden deaths in sickle cell diseases. 2023.
21. Kanter, J., et al., Biologic and clinical efficacy of LentiGlobin for sickle cell disease. 2022. 386(7): p. 617-628.
Article Sidebar
Article Details
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
All articles published in JPTCP are licensed under Copyright Creative Commons Attribution-NonCommercial 4.0 International License.
Dr Muhammad Tariq Hamayun Khan
Assistant Professor, Department of Hematology, Pathology Department, Burns & Plastic Surgery Center Hayatabad, Peshawar, Pakistan
Dr Asma Shah
Lecturer, Department of Pathology (Hematology), Women Medical College, Abbottabad, Pakistan
Aliza Ali khan
Lecturer, Department of Pharmacy, Qurtuba University, Dera Ismail Khan, Pakistan
Dr Muhammad Arif Jan
Junior Registrar, Department of Pediatrics Medicine, Saidu Group of Teaching Hospital, Swat, Pakistan
Dr Hina Umair
Assistant Professor, Department of Physiology, Wah Medical College, NUMS, Wah Cantt, Pakistan
Dr Tania Khattak
Assistant Professor, Department of Pathology (Histopathology), Nowshera Medical College, Nowshera, Pakistan