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 (e.g. 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.

Presentation

• Left upper quadrant (LUQ) mass or 'uncomfortable' abdominal pain; early satiety from compressed stomach.
• Pancytopenia due to hypersplenism (see 'Hypersplenism' heading, below).

Examination

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.

Investigations

Investigation will depend on the specific clinical presentation of the patient but may include:
• Abdominal ultrasound imaging, MRI scanning, CT scanning.
• FBC, reticulocytes, blood film.
• Haemoglobinopathy screen.
• LFTs.
• Virology, microbiology.
• Serum protein electrophoresis.
• Peripheral blood cell markers (leukaemia, lymphoma).
• Radioisotope liver and spleen scan.¹
• Liver biopsy, bone marrow biopsy, lymph node biopsy.

Aetiology

• Haematological:
  • Haemolytic anaemias (e.g. thalassaemia, red cell defects, sickle cell anaemia).
  • Acute leukaemias, chronic leukaemias.
  • Polycythaemia rubra vera.
  • Macroglobulinaemia.
  • Lymphoma (Hodgkin's disease and non-Hodgkin's lymphoma).
  • Essential thrombocythaemia.
  • Myelofibrosis.
• Infections:
  • Malaria.
  • Schistosomiasis.
  • Visceral leishmaniasis (Kala-azar).
  • Tuberculosis, brucellosis.
  • Glandular fever, viral hepatitis.
  • Infective endocarditis.
• Tumours and cysts:
  • Splenic abscesses.
  • Splenic metastases.
  • Cysts, e.g. hydatid, dermoid.
  • Tumours, e.g. haemangioma.
• Congestive splenomegaly:
  • Liver cirrhosis.
  • Budd-Chiari syndrome.¹
  • 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.
  • Amyloidosis.

Causes of massive splenomegaly

• Chronic myeloid leukaemia.
• Myelofibrosis, malaria (hyper-reactive malarial splenomegaly).
• Leishmaniasis.
• 'Tropical splenomegaly' (idiopathic; Africa, South-east Asia).
• Gaucher's syndrome.¹

Splenomegaly in children

This is most commonly caused by infection, autoimmune disorders or haemolysis. It may be a presenting feature of neoplasia (e.g. 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.

Hypersplenism

• 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.

Management

• Treatment of the cause.
• Blood transfusions may be required.
• Open or laparoscopic splenectomy may be indicated to control or stage the disease (e.g. hereditary spherocytosis, Hodgkin's disease).
• Patients with impaired splenic function need prophylactic vaccinations etc. (see separate article Splenectomy, Hyposplenism and Asplenia).

Document references

1. Pozo AL, Godfrey EM, Bowles KM; Splenomegaly: investigation, diagnosis and management. Blood Rev. 2009 May;23(3):105-11. Epub 2008 Dec 4. [abstract]

Internet and further reading

• Kinirons M, Ellis H; Splenomegaly; French's Index of Differential Diagnosis; 14th Edition (2005)

Acknowledgements