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The spleen is involved in producing protective humoral antibodies, the production and maturation of B and T cells and plasma cells, removal of unwanted particulate matter (eg, bacteria) and it also acts as a reservoir for blood cells, especially white cells and platelets. When the spleen is palpable it has usually reached at least twice its normal size.
- Left upper quadrant (LUQ) mass or 'uncomfortable' abdominal pain; early satiety from compressed stomach.
- Pancytopenia due to hypersplenism (see 'Hypersplenism' heading, below).
When considering whether an LUQ mass is an enlarged spleen, features of an enlarged spleen include that:
- It moves with respiration.
- It enlarges towards the right iliac fossa (RIF) - always start palpation in the RIF and move across towards the right upper quadrant (or a massive splenomegaly may be missed).
- You cannot palpate above it - the upper margin lies under the ribs.
- You may feel a notch.
- It is dull to percussion.
Always remember to check for any accompanying lymphadenopathy and/or features of liver disease.
- Liver biopsy.
- Bone marrow biopsy.
- Lymph node biopsy.
- Haemolytic anaemias (eg, thalassaemia, red cell defects, sickle cell anaemia).
- Acute leukaemias, chronic leukaemias.
- Polycythaemia rubra vera.
- Lymphoma (Hodgkin's disease and non-Hodgkin's lymphoma).
- Essential thrombocythaemia.
- Visceral leishmaniasis (Kala-azar).
- Tuberculosis, brucellosis.
- Glandular fever, viral hepatitis.
- Infective endocarditis.
- Tumours and cysts:
- Splenic abscesses.
- Splenic metastases.
- Cysts - eg, hydatid, dermoid.
- Tumours - eg, haemangioma.
- Congestive splenomegaly:
- Liver cirrhosis.
- Budd-Chiari syndrome.
- Portal or splenic vein obstruction.
- Heart failure.
- Connective tissue disorders:
- Systemic lupus erythematosus.
- Felty's syndrome.
- Other disorders:
- Gaucher's disease.
- Niemann-Pick disease.
- Histiocytosis X.
Causes of massive splenomegaly
- Chronic myeloid leukaemia.
- Myelofibrosis, malaria (hyper-reactive malarial splenomegaly).
- 'Tropical splenomegaly' (idiopathic; Africa, Southeast Asia).
- Gaucher's disease.
Splenomegaly in children
This is most commonly caused by infection, autoimmune disorders or haemolysis. It may be a presenting feature of neoplasia (eg, metastatic neuroblastoma). Causes include:
- Infection: glandular fever, cytomegalovirus (CMV), other viral infections, often accompanied by lymphadenopathy; bacterial, protozoal, and fungal infections.
- Autoimmune: juvenile rheumatoid arthritis.
- Haemolysis: hereditary spherocytosis, sickle cell anaemia, thalassaemia.
- Neoplasia: acute lymphoblastic leukaemia (ALL), Hodgkin's disease and non-Hodgkin's lymphoma (NHL), acute or chronic myeloblastic leukaemia, neuroblastoma.
- Inherited diseases: Gaucher's disease and other storage disorders.
- This is a pancytopenia occurring in patients with an enlarged spleen. It is due to large numbers of cells being pooled and destroyed in the spleen's reticulo-endothelial system, and haemodilution because of an increased plasma volume.
- It can present with symptoms of anaemia, infection, or bleeding.
- Bone marrow biopsy shows normal or hyperplastic marrow.
- Splenic sequestration crisis may develop in young children with sickle cell anaemia, which can precipitate hypovolaemic shock and death, and is an indication for splenectomy.
- Treatment of the cause.
- Blood transfusions may be required.
- Open or laparoscopic splenectomy may be indicated to control or stage the disease (eg, hereditary spherocytosis, Hodgkin's disease).
- Patients with impaired splenic function need prophylactic vaccinations, etc (see separate article Splenectomy and Hyposplenism).
Further reading and references
Pozo AL, Godfrey EM, Bowles KM; Splenomegaly: investigation, diagnosis and management. Blood Rev. 2009 May23(3):105-11. Epub 2008 Dec 4.