A Single-Centre Study of Splenic Dysfunction in Children with Sickle Cell Disease in Central India

Chronic hemolytic anemia, also known as sickle cell disease (SCD) is linked to episodic acute complications that progressively affects spleen, with evidence of hyposplenism reported even in infants. Young children with SCD frequently exhibit sequestration crisis, splenomegaly, and increased splenic red pulp activity, which may coexist with loss of function. The current study was performed to investigate the frequency and determinants of splenic dysfunction in children with SCD.

This cross-sectional study was carried out from June 2019 to December 2020, involving children aged 1 to 15 years with SCD who were screened for splenic dysfunction. Exclusion criteria included children who had undergone splenectomy, and those with other conditions impacting splenic function, such as congenital malformations, immunodeficiencies, and chronic diseases like tuberculosis, nephrotic syndrome, diabetes mellitus, chronic liver disease, celiac disease, or malignancy. Clinical examination and ultrasonography were used to evaluate the splenic size while autologous RBCs tagged with Technetium-99m (99mTc) and the existence of Well Jolly bodies in the peripheral smear were used to evaluate splenic dysfunction. Multiple logistic regression was used to analyse laboratory and clinical predictors of splenic dysfunction.

The results showed the following findings:

• The study enrolled 66 children with SCD, with an average (SD) age of 7.41 (3.3) years. 
• Impaired and absent splenic function, as determined by 99mTc scintigraphy, were found in 13 (19.7%) and 3 (4.6%) children, respectively.
• Howell Jolly bodies in the peripheral smear were observed in 5 (7.5%) children; 3 of these had abnormal scintigraphy results, and all 5 had splenomegaly.
• Independent predictors of splenic dysfunction included age over 5 years, more than 4 episodes of vaso-occlusive crisis (VOC), over 3 hospitalizations in the past, more than 5 blood transfusions, lack of hydroxyurea treatment, reticulocyte count above 4%, and HbS levels over 70%.

To conclude, as compared to Western nations, Central India has a lower rate of splenic dysfunction in children with SCD. Moreover, it is feasible to customize the choice to initiate antibiotic prophylaxis for affected kids.

Source: Johns J, Goel AK, Jondhale S, Venkatesan DK, Ravina M, Shah S, Syal S. Splenic Dysfunction in Children With Sickle Cell Disease: A Single Centre Experience From Central India. Indian Pediatr. 2024 Sep 15;61(9):817-822. Epub 2024 Jun 20. PMID: 38910365.