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Splenomegaly and Hypersplenism

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 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 below).

Examination

When considering whether a LUQ mass is an enlarged spleen, features of an enlarged spleen include:

  • Moves with respiration
  • Enlarges towards the right iliac fossa (RIF) - always start palpation in the RIF and move across towards the RUQ or you may miss massive splenomegaly!
  • You cannot palpate above it - 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
Aetiology

Causes of massive splenomegaly

  • CML
  • Myelofibrosis, malaria (hyper-reactive malarial splenomegaly)
  • Leishmaniasis
  • 'Tropical splenomegaly' (idiopathic; Africa, SE Asia)
  • Gaucher's syndrome

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, 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: ALL, Hodgkin disease and NHL, acute or chronic myeloblastic leukemia, 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 reticuloendothelial 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
  • Treat cause.
  • 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 on splenectomy and hyposplenism).


Internet and Further Reading
  • Kinirons M, Ellis H; Splenomegaly; French's Index of Differential Diagnosis; 14th Edition (2005)
Acknowledgements EMIS is grateful to Dr Colin Tidy for writing this article. The final copy has passed scrutiny by the independent Mentor GP reviewing team. ©EMIS 2007.
DocID: 1099
Document Version: 20
DocRef: bgp1069
Last Updated: 21 Aug 2006
Review Date: 20 Aug 2008


















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PS - Health and Poverty

Perhaps the biggest cause of ill health in the world is poverty. Help to Make Poverty History. For example, why not lend some of your money to disadvantaged communities to enable them to trade their way out of poverty through schemes such as Shared Interest.

See also MAKEPOVERTYHISTORY North East for details and links to campaigns against poverty.

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