Considering the spleen in sickle cell disease

Area of Science:

• Hematology
• Pediatric Medicine
• Physiology

Background:

• The spleen's role in human physiology is often underestimated.
• In sickle cell disease (SCD), splenic dysfunction is a primary driver of severe, life-threatening complications, especially in early childhood.
• Progressive splenic decline and anatomical disappearance occur in SCD, influenced by genetic and environmental factors.

Purpose of the Study:

• To review spleen structure and function.
• To emphasize the spleen's critical role in sickling disorders.
• To detail clinical features, complications, and therapeutic options for spleen damage in SCD.

Main Methods:

• Literature review of spleen structure and function.
• Analysis of clinical features of spleen damage in sickle cell disease.
• Review of short- and long-term consequences and therapeutic strategies.

Main Results:

• Spleen dysfunction in SCD leads to high risks of pneumococcal sepsis and acute splenic sequestration crisis in children.
• The spleen undergoes functional decline and anatomical loss throughout the disease course.
• Variability in spleen changes is modulated by genetic and environmental factors.

Conclusions:

• Understanding spleen structure and function is crucial for managing sickling disorders.
• Clinical studies are urgently needed to guide management of recurrent splenic sequestration and splenectomy timing in children with sickling disorders.