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Splenic Dysfunction in Children With Sickle Cell Disease: A Single Centre Experience From Central India
Juliet Johns1, Anil Kumar Goel2, Sunil Jondhale1
1Department of Pediatrics, All India Institute of Medical Sciences, Raipur, Chhattisgarh, India.
Insights
In children with sickle cell disease (SCD), splenic dysfunction is less common in Central India than in the West. Key predictors include older age, frequent crises, hospitalizations, and specific lab values.
Area of Science:
- Pediatrics
- Hematology
- Medical Imaging
Background:
- Sickle cell disease (SCD) frequently leads to splenic dysfunction in children.
- Early identification of splenic dysfunction is crucial for managing SCD complications.
- Prevalence and predictors of splenic dysfunction in Central India require specific investigation.
Purpose of the Study:
- To determine the prevalence of splenic dysfunction in pediatric SCD patients in Central India.
- To identify clinical and laboratory predictors associated with splenic dysfunction in this population.
Main Methods:
- A cross-sectional study included 66 children (1-15 years) with SCD.
- Splenic function was assessed using Technetium-99m labeled autologous RBC scintigraphy and Howell Jolly bodies.
- Exclusion criteria targeted conditions known to affect splenic function.
Main Results:
- 19.7% had impaired splenic function and 4.6% had absent splenic function via scintigraphy.
- Howell Jolly bodies were present in 7.5% of children, all with splenomegaly.
- Independent predictors of splenic dysfunction included age > 5 years, >4 vaso-occlusive crises, >3 hospitalizations, >5 blood transfusions, no hydroxyurea, high reticulocyte count, and high HbS levels.
Conclusions:
- Splenic dysfunction prevalence in Central Indian children with SCD is lower than reported in Western studies.
- Findings suggest individualized approaches to antibiotic prophylaxis may be appropriate for these children.
Objective:
To assess the prevalence and predictors of splenic dysfunction in children with sickle cell disease (SCD).
Methods:
A cross-sectional study was conducted between June 2019 and December 2020 where children aged 1 to 15 years of age with SCD were screened for splenic dysfunction. Children who were splenectomised, those with other diseases known to affect splenic function like congenital malformations, immunodeficiencies, and chronic diseases like tuberculosis, nephrotic syndrome, diabetes mellitus, chronic liver disease, celiac disease or malignancy were excluded. Splenic size was assessed by clinical examination and ultrasonography. Splenic dysfunction was assessed by Technetium-99m (99mTc) labeled autologous RBCs and by the presence of Howell Jolly bodies in the peripheral smear. Laboratory and clinical predictors of splenic dysfunction were assessed by multiple logistic regression.
Results:
We evaluated 66 children with SCD with a mean (SD) age of 7.41 (3.3) years. Impaired and absent splenic function as assessed by 99mTc scintigraphy was found in 13 (19.7%), and 3 (4.6%) children, respectively. Howell Jolly bodies in peripheral smear were found in 5 (7.5%) children; 3 of them had abnormal uptake on scintigraphy; all five had splenomegaly. Age > 5 years, > 4 episodes of vaso-occlusive crisis (VOC), > 3 hospitalization events in the past, > 5 blood transfusions, children not receiving hydroxyurea, reticulocyte count > 4%, and HbS > 70% were independent predictors of splenic dysfunction.
Conclusion:
The prevalence of splenic dysfunction in children with SCD in Central India is lower than that reported from the West. The decision to start antibiotic prophylaxis can be individualized in these children.
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