syndrome characterized by splenomegaly and a cytopenia (anaemia, leukopenia, thrombocytopenia or pancytopenia). Not all patients with splenomegaly have hypersplenism.

<aside> 💡 TLDR

• spleen gets bigger = does more work. double in sisze = double RBC destruction
  • anemia
  • leukopenia
  • thrombocytopenia </aside>

Aetiology:

• Primary: Idiopathic/rare

• Secondary: Underlying disease causes splenomegaly resulting in hypersplenism

  

Pathophysiology:

• Retention of cells in the spleen: As the spleen enlarges the proportion of hematopoietic cells increases (up to 20x the normal amount). These cells are subsequently phagocytosed by splenic macrophages resulting in peripheral cytopenia.
• Enhanced macrophage phagocytosis: It is believed that some patients with splenomegaly (particularly caused by portal hypertension) have significantly increased macrophage counts and overactive macrophages.

Clinical Features

• Features of underlying cause
• Splenomegaly
• Anemia: pallor, fatigue, dyspnea, palpitations
• Thrombocytopenia: bleeding, bruising
• Leukopenia: increased risk of infections

Investigations