Condition in which spleen removes circulating RBCs, granulocytes and platelets in excess quantity.
Diagnosis:
- Pancytopenia
- Normal or hypercellular bone marrow
- Splenomegaly
- Correction of cytopenias after splenectomy
Classification
- Primary – when no aetiology for enlarged spleen is found.
- Secondary –
- Portal hypertension
- Infiltrative disease, lymphoma, myelofibrosis
- Hemolyitc anaemias, hematological disorders
- Rheumatoid arthritis (Felty’s syndrome)
- Tropical splenomegaly syndrome
Pathogenesis
When splenic size increases, there is increases pooling of blood in an environment with relatively reduced availability of nutrients but full of phagocytes.
This leads to exaggerated sequestration and destruction of cells leading to
- Pancytopenia
- Hemolysis
- Increased plasma volume
Treatment
Therapy needed when cytopenias become severe and symptomatic
Treatment of underlying causes
Splenectomy if underlying cause cannot be corrected or treated