[Splenic dysfunction in sickle cell disease: An update]

Area of Science:

• Immunology
• Hematology
• Physiology

Context:

• The spleen is vital for blood filtration and immune defense, particularly against encapsulated bacteria.
• Impaired splenic function (hyposplenism) or splenectomy increases infection susceptibility and thrombosis risk.
• Sickle cell disease frequently causes functional asplenia early in life due to red blood cell damage.

Purpose:

• To provide an updated overview of spleen physiology and function.
• To elucidate the pathophysiology of spleen damage in sickle cell disease.
• To enhance understanding of spleen-related complications in sickle cell disease.

Summary:

• The spleen's red pulp clears old red blood cells, while the white pulp defends against encapsulated bacteria.
• Hyposplenism and splenectomy lead to increased risks of infections, thrombosis, and pulmonary hypertension.
• Sickle cell disease causes early functional asplenia, with risks of hypersplenism and acute splenic sequestration.

Impact:

• Improved understanding of spleen's role in immunity and blood homeostasis.
• Better comprehension of sickle cell disease pathophysiology and its splenic complications.
• Potential for improved management strategies for patients with sickle cell disease and splenic dysfunction.